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1.
J Card Surg ; 35(11): 2927-2933, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33111442

RESUMO

OBJECTIVE: The arterial switch operation (ASO) is the standard treatment for the transposition of the great arteries. The timely variation in the residual pressure gradient across the pulmonary arteries is ill-defined. This study is aimed to study the progressive changes in the pressure gradient across the pulmonary valve and pulmonary arteries after ASO. METHODS: All eligible patients for this study who underwent ASO between 2000 and 2019 were reviewed. Transthoracic echocardiography was used to estimate the peak pressure gradient across the pulmonary artery and its branches. The primary outcome was the total peak pressure gradient (TPG) which is the sum of peak pressure gradients across the main pulmonary artery and pulmonary artery branches. Furthermore, longitudinal data analyses with mixed-effect modeling were used to determine the independent predictors for the changes in the pressure gradient. RESULTS: Three hundred and nine patients were included in the study. Over a 17-year follow-up, the freedom from pulmonary stenosis reintervention was 95% (16 out of the 309 patients underwent reintervention = 5%). The longitudinal data analyses of serial 1844 echocardiographic studies for the included patients revealed that the TPG recorded in the first postoperative echocardiogram across pulmonary valve, right and left pulmonary artery branches was the most significant predictor for reintervention. CONCLUSION: The total peak gradient measured in the first postoperative echocardiogram is the most important predictor for reintervention. We propose that a total peak gradient in the first postoperative echocardiography of 55 mm Hg or more is a predictor for reintervention.


Assuntos
Pressão Arterial , Transposição das Grandes Artérias/métodos , Artéria Pulmonar/fisiopatologia , Valva Pulmonar/fisiopatologia , Transposição dos Grandes Vasos/fisiopatologia , Transposição dos Grandes Vasos/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Ecocardiografia , Feminino , Seguimentos , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/fisiopatologia , Valor Preditivo dos Testes , Artéria Pulmonar/diagnóstico por imagem , Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia , Reoperação , Transposição dos Grandes Vasos/diagnóstico por imagem , Resultado do Tratamento , Adulto Jovem
2.
Int Heart J ; 61(5): 1075-1078, 2020 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-32921664

RESUMO

Pulmonary valve stenosis (PVS) accounts for approximately 10% of all congenital heart defects. Echocardiography and right heart catheterization are the gold standards for diagnosis of PVS and for assessing disease severity and responsiveness to treatment.Recently, cardiac magnetic resonance imaging (cMRI) has been established as an important tool to comprehensively evaluate cardiac structure and function; however, research into the usefulness of cMRI for PVS management is limited. Here, we describe a case of a 59-year-old female with isolated, severe PVS who was successfully treated with balloon pulmonary valvuloplasty (BPV) followed by sequential cMRI at 1 and 12 months. Exertional dyspnea and elevated plasma BNP concentration were observed 1 month after BPV; however, echocardiographic findings did not indicate recurrent stenosis or increased pulmonary valve regurgitation but an increase in mitral E/e'. cMRI demonstrated improved systolic forward flow and RV function with enlargement of LV volume, and the rapid increase in LV preload might be associated with the transient deterioration in symptoms and BNP level, which both gradually improved within 3 months after BPV. cMRI further depicted that a reduced RV mass index and increased RV cardiac output were achieved gradually during the follow-up period.In conclusion, cMRI in combination with echocardiography was sufficiently informative to follow-up this PVS patient both before and after BPV. cMRI is easily reproducible in adult patients; therefore, cMRI should be recommended for long-term follow-up in adult PVS patients.


Assuntos
Valvuloplastia com Balão/métodos , Ecocardiografia , Imagem por Ressonância Magnética , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Recuperação de Função Fisiológica , Técnicas de Imagem Cardíaca , Débito Cardíaco , Dispneia/fisiopatologia , Feminino , Humanos , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/sangue , Período Pós-Operatório , Estenose da Valva Pulmonar/congênito , Estenose da Valva Pulmonar/fisiopatologia , Índice de Gravidade de Doença , Função Ventricular Direita
3.
PLoS One ; 15(8): e0237193, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32780780

RESUMO

BACKGROUND: The myocardial adaptive mechanism in patients with repaired tetralogy of Fallot (rTOF) is less understood. We aimed to investigate biventricular myocardial adaptive remodeling in rTOF patients. METHODS: We recruited 32 rTOF patients and 38 age- and sex-matched normal controls. The pulmonary stenosis of rTOF patients was measured using catheterized pressure gradient between right ventricle (RV) and pulmonary artery (PGRVPA). rTOF patients with PGRVPA < 15 mmHg and ≥15 mmHg were classified as low pulmonary stenosis (rTOFlow, n = 19) and high pulmonary stenosis (rTOFhigh, n = 13) subgroups, respectively. Magnetic resonance imaging tissue phase mapping was employed to evaluate the voxelwise biventricular myocardial motion in longitudinal (Vz), radial (Vr), and circumferential (Vφ) directions. RESULTS: The rTOFlow subgroup presented higher pulmonary regurgitation fraction than rTOFhigh subgroup (p < 0.001). Compared with the normal group, only rTOFlow subgroup presented a decreased RV ejection fraction (RVEF) (p < 0.05). The rTOFlow subgroup showed decreased systolic and diastolic Vz in RV and LV, whereas rTOFhigh subgroup showed such change only in RV. In rTOFlow subgroup, RVEF significantly correlated with RV systolic Vr (r = 0.56, p < 0.05), whereas LVEF correlated with LV systolic Vz (r = 0.51, p = 0.02). Prolonged QRS correlated with RV systolic Vr (r = -0.58, p < 0.01) and LV diastolic Vr (r = 0.81, p < 0.001). No such correlations occurred in rTOFhigh subgroup. CONCLUSIONS: The avoidance of unfavorable functional interaction in RV and LV in rTOFhigh subgroup suggested that adequate pulmonary stenosis (PGRVPA ≥ 15 mmHg in this sereis) has a protective effect against pulmonary regurgitation.


Assuntos
Imagem por Ressonância Magnética/métodos , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/reabilitação , Adulto , Pressão Sanguínea , Estudos de Casos e Controles , Teste de Esforço , Feminino , Frequência Cardíaca , Humanos , Masculino , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Volume Sistólico , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Esquerda , Função Ventricular Direita , Adulto Jovem
4.
J Thorac Cardiovasc Surg ; 160(2): 476-484, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32014323

RESUMO

OBJECTIVES: The long-term durability of the 2 most commonly used types of bioprosthetic valves in the pulmonic position, the porcine and pericardial valves, is unclear. We compared the long-term durability of the pericardial (Carpentier-Edwards PERIMOUNT [CE]) and porcine (Hancock II) valves in the pulmonic position in patients with congenital cardiac anomalies. METHODS: We retrospectively reviewed the medical records of 258 cases (248 patients) of pulmonary valve implantation or replacement using CE (129 cases, group CE) or porcine (129 cases, group H) valves from 2 institutions between 2001 and 2009. RESULTS: The patients' age at pulmonary valve implantation was 14.9 ± 8.7 years. No significant differences in perioperative characteristics were observed between groups CE and H. Follow-up data were complete in 219 cases (84.9%) and the median follow-up duration was 10.5 (interquartile range, 8.4∼13.0) years. Ten mortalities (3.9%) occurred. Sixty-four patients underwent reoperation for pulmonary valve replacement due to prosthetic valve failure; 10 of these 64 patients underwent reoperation during the study period. Patients in group CE were significantly more likely to undergo reoperation (hazard ratio, 2.17; confidence interval, 1.26-3.72; P = .005) than patients in group H. Patients in group CE showed had a greater prosthetic valve dysfunction (moderate-to-severe pulmonary regurgitation or pulmonary stenosis with ≥3.5 m/s peak velocity through the prosthetic pulmonary valve) rate (hazard ratio, 1.83; confidence interval, 1.07-3.14; P = .027) than patients in group H. CONCLUSIONS: Compared with the pericardial valve, the porcine valve had long-term advantages in terms of reduced reoperation rate and prosthetic valve dysfunction in the pulmonic position in patients with congenital cardiac anomalies.


Assuntos
Bioprótese , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Adolescente , Animais , Bovinos , Criança , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/mortalidade , Hemodinâmica , Xenoenxertos , Humanos , Masculino , Complicações Pós-Operatórias/cirurgia , Desenho de Prótese , Falha de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/mortalidade , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/mortalidade , Estenose da Valva Pulmonar/fisiopatologia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Seul , Sus scrofa , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
6.
Ann Thorac Surg ; 109(4): 1127-1131, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31518586

RESUMO

BACKGROUND: Limited data exist on long-term pulmonary valve function after the Ross procedure. This study sought to determine the long-term function of the pulmonary valve in 443 consecutive adult patients who underwent a Ross procedure. METHODS: All 443 patients who underwent a Ross procedure between November 1992 and March 2018 were reviewed retrospectively. All underwent pulmonary valve replacement using a cryopreserved pulmonary allograft. Freedom from the study's outcomes were calculated using Kaplan Meier survival. Risk factors for valve failure were analyzed using Cox regression. RESULTS: Mean age at time of operation was 39 years (range: 15-66 years). There was 1 (0.2%, 1 of 443) operative mortality. Nine patients required reintervention on the pulmonary allograft at a mean 6.1 years (range: 1-12 years) after Ross procedure. Patients required pulmonary allograft reintervention for infective endocarditis (n = 4), severe pulmonary stenosis (n = 4), or severe pulmonary regurgitation (n = 1). Freedom from pulmonary allograft reintervention was 98.9% (95% confidence interval [CI] 97.1%-99.6%), 97.7% (95% CI 95.1%-98.9%), 96.6% (95% CI 93.3%-98.3%), and 96.6% (95% CI 93.3%-98.3%) at 5, 10, 15, and 20 years, respectively. Freedom from pulmonary allograft dysfunction (at least moderate pulmonary regurgitation and/or mean systolic gradient ≥ 25 mm Hg and/or reintervention) was 94.5% (95% CI 91.6%-96.4%), 88.1% (95% CI 83.6%-91.4%), 84.9% (95% CI 79.6%-88.9%), and 78.3% (95% CI 69.5%-84.9%) at 5, 10, 15, and 20 years, respectively. No risk factors were identified to influence pulmonary valve durability. CONCLUSIONS: The pulmonary valve allograft gives excellent long-term function when used in adults undergoing the Ross procedure. Reintervention on the pulmonary valve is rare and significant pulmonary allograft dysfunction is uncommon.


Assuntos
Implante de Prótese de Valva Cardíaca/métodos , Ventrículos do Coração/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Função Ventricular Direita/fisiologia , Adolescente , Adulto , Idoso , Ecocardiografia sob Estresse/métodos , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Período Pós-Operatório , Desenho de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico , Estenose da Valva Pulmonar/fisiopatologia , Reoperação , Estudos Retrospectivos , Transplante Autólogo , Adulto Jovem
7.
J Thorac Cardiovasc Surg ; 159(3): 1063-1071.e1, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31400815

RESUMO

BACKGROUND: Pulmonary valve replacement is very common among patients with congenital heart disease. The Carpentier Edwards Perimount valve (Edwards Lifesciences, Irvine, Calif), which was originally designed for the aortic position is among the most implanted valves. We aim to describe the follow-up of this valve in the pulmonary position. METHODS: Patients with a Perimount valve implanted between 2003 and 2013 in the University Medical Center Groningen were followed for the primary end point reintervention, defined as surgical or transcatheter valve replacement. Secondary end point was the occurrence of valve failure, defined as significant valvular regurgitation or stenosis. Explanted valves were histologically examined. RESULTS: Forty-five patients (median age at operation 27.8 years, 55.6% women) had a mean follow-up duration of 5.8 ± 3.3 years. There were 7 reinterventions (5 surgical and 2 transcatheter). Freedom from reintervention was respectively 95% ± 4% and 83% ± 8% at 5- and 10- years of follow-up. Freedom from valve failure was 75% ± 4% at 2 years, 65% ± 8% at 5 years of follow-up and 57% ± 10% at 10 years of follow-up. Morphology evaluation (n = 4) showed stiffened valves in the open position, with extensive fibrous tissue overgrowth on the leaflets and a variable proliferation of myofibroblasts. CONCLUSIONS: The Perimount valve has adequate function in the pulmonary valve position at 5 years of follow-up, although after 10 years of follow-up valve failure and reinterventions are common. Explanted valves show retraction and stiffening of the leaflets due to a fibrotic layer on both sides of the leaflet.


Assuntos
Bioprótese , Implante de Prótese de Valva Cardíaca/instrumentação , Próteses Valvulares Cardíacas , Pericárdio/transplante , Valva Pulmonar/cirurgia , Stents , Adolescente , Adulto , Cateterismo Cardíaco , Remoção de Dispositivo , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Xenoenxertos , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos , Intervalo Livre de Progressão , Desenho de Prótese , Falha de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/etiologia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/cirurgia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Adulto Jovem
8.
Turk Kardiyol Dern Ars ; 47(7): 619-621, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31582677

RESUMO

Pulmonary valvular stenosis is a rare valvular disease; it accounts for 7% to 12% of all patients with congenital heart disease. Patients with mild or moderate pulmonary stenosis (PS) are usually asymptomatic and the stenosis is often detected incidentally with echocardiography performed for another reason. Severe PS typically presents with symptoms related to increased right ventricular pressure and right heart failure. Valvular heart diseases are associated with increased morbidity and mortality in pregnancy due to cardiovascular alterations that occur during the gestational period, such as increased extracellular volume, a faster heart rate, and decreased venous return due to compression of the vena cava inferior by the enlarged uterus. While mild or moderate PS can be well tolerated in pregnancy, severe PS can lead to maternal and fetal perinatal complications. Presently described is the case of a pregnant patient with severe PS who successfully underwent balloon valvuloplasty in the third trimester.


Assuntos
Complicações Cardiovasculares na Gravidez/diagnóstico , Diagnóstico Pré-Natal , Estenose da Valva Pulmonar/diagnóstico , Valvuloplastia com Balão , Diagnóstico Diferencial , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Gravidez , Complicações Cardiovasculares na Gravidez/fisiopatologia , Complicações Cardiovasculares na Gravidez/terapia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/terapia , Índice de Gravidade de Doença , Gravação em Vídeo , Adulto Jovem
9.
Catheter Cardiovasc Interv ; 94(4): 625-635, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31471941

RESUMO

OBJECTIVES: We sought to delineate the risk factors for infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TCPVR). BACKGROUND: Despite the therapeutic benefits of TCPVR for treatment of dysfunctional right ventricular outflow tracts, IE is a major complication of the approach. Specific hemodynamic gradients and patient immune status as predisposing factors for IE are largely unexplored. METHODS: We performed a retrospective review of patients who had undergone TCPVR at UCLA between October 2010 and October 2017. Cases of IE were diagnosed based on the modified Duke criteria. RESULTS: Two hundred and thirty-five cases of TCPVR were performed with a mean follow-up of 2.6 years (range 0.0-8.0 years). Sixteen distinct IE events developed in 13 patients (Melody™ n = 12, SAPIEN n = 1), with a median time from implant to IE of 3.3 years (range 2.0-7.2 years). Univariate Cox regression showed that immunocompromised status was significantly associated with the development of IE hazard ratios (HR 5.43 [1.80-16.4], p = .003). Kaplan-Meier curves show that the 5-year freedom from IE among immunocompetent patients was 87% (95% CI 78-96%) versus 64% (95% CI 39-89%) among immunocompromised patients (log-rank p = .02). Postimplant right ventricular systolic pressure was higher among immunocompromised patients (p = .03). The risk of IE post-TCPVR in immunocompromised patients with residual pulmonary stenosis was 43%. CONCLUSIONS: Among the risk factors examined in this study, immunocompromised status was the most significant predictor of IE development post-TCPVR. Patients with the lowest risk of IE are those with competent immune systems, without a history of IE, and with minimal residual pulmonary valve gradients post-TCPVR.


Assuntos
Cateterismo Cardíaco/efeitos adversos , Endocardite/etiologia , Cardiopatias Congênitas/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Próteses Valvulares Cardíacas/efeitos adversos , Infecções Relacionadas à Prótese/etiologia , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Adolescente , Adulto , Cateterismo Cardíaco/instrumentação , Tomada de Decisão Clínica , Endocardite/diagnóstico , Endocardite/imunologia , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/imunologia , Cardiopatias Congênitas/fisiopatologia , Implante de Prótese de Valva Cardíaca/instrumentação , Hemodinâmica , Humanos , Hospedeiro Imunocomprometido , Masculino , Intervalo Livre de Progressão , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/imunologia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/imunologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/imunologia , Estenose da Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Adulto Jovem
11.
Catheter Cardiovasc Interv ; 94(3): 409-413, 2019 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-31408262

RESUMO

BACKGROUND: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT). METHODS: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath. RESULTS: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access. CONCLUSIONS: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs.


Assuntos
Cateterismo Cardíaco/instrumentação , Cateteres Cardíacos , Implante de Prótese de Valva Cardíaca/instrumentação , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Stents , Adolescente , Adulto , Idoso , Cateterismo Cardíaco/efeitos adversos , Criança , Colorado , Feminino , Implante de Prótese de Valva Cardíaca/efeitos adversos , Hemodinâmica , Humanos , Irlanda , Itália , Masculino , Pessoa de Meia-Idade , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia , Recuperação de Função Fisiológica , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
12.
Methodist Debakey Cardiovasc J ; 15(2): 122-132, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384375

RESUMO

Right ventricular outflow tract (RVOT) dysfunction is common following surgical repair of tetralogy of Fallot and other forms of complex congenital heart disease. This results in pulmonary stenosis or regurgitation and may ultimately lead to RV failure and dysrhythmias. Transcatheter valve technologies are now available to treat certain patients with RVOT dysfunction. Current devices include the Medtronic Melody valve and the Edwards Lifesciences SAPIEN XT. Although these valves are approved for use in dysfunctional circumferential RVOT conduits, they are increasingly being used off label for nonconduit outflow tracts. Procedural complications include but are not limited to conduit rupture and coronary compression. Longer-term complications include stent fracture and endocarditis. Outcomes with these valves have demonstrated durable relief of stenosis and regurgitation. The Medtronic Harmony valve and the Alterra Prestent from Edwards Lifesciences are investigational devices that are intended to treat the patulous RVOT that is too large to accommodate currently available valves. This review will focus on current indications to treat RVOT dysfunction, existing transcatheter valve technologies, and investigational devices undergoing clinical trials. Hopefully, within the not-too-distant future, transcatheter pulmonary valve implantation will be feasible in the vast majority of patients with RVOT dysfunction following surgical repair of congenital heart disease.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Implante de Prótese de Valva Cardíaca , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Hemodinâmica , Humanos , Complicações Pós-Operatórias/etiologia , Desenho de Prótese , Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/etiologia , Estenose da Valva Pulmonar/fisiopatologia , Fatores de Risco , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/fisiopatologia
13.
Endocr J ; 66(11): 983-994, 2019 Nov 28.
Artigo em Inglês | MEDLINE | ID: mdl-31292302

RESUMO

Noonan syndrome (NS) is a heterogeneous disorder with multiple congenital malformations. Recent advances in molecular and genetic approaches have identified a number of responsible genes for NS, most of which are components of the RAS/MAPK signaling pathway, and genotype-phenotype correlation analyses have been extensively performed; however, analysis of Japanese NS patients is limited. Here, we evaluated clinical characteristics in genetically diagnosed NS patients and their relationships to genotypes. A total of 48 clinically diagnosed NS were included, and responsible mutations were identified in 39 patients (81.3%) with PTPN11 mutations being the most prevalent followed by SOS1 mutations. Cardiac anomalies including pulmonary stenosis and hypertrophic cardiomyopathy were most prevalent (87.2%), and the prevalence of hypertrophic cardiomyopathy was greater in patients without PTPN11 mutations than in those with PTPN11 mutations. Short stature was the second-most prevalent (69.2%) characteristic, and present height SD score was significantly associated with height SD score at 1 year old. Patients with SOS1 mutations had greater present height SD score and better growth during infancy. These findings suggest the presence of a genotype-phenotype correlation in Japanese patients with NS, which enables us to use genetic information to predict the clinical course and may allow for genotype-based medical interventions.


Assuntos
Cardiomiopatia Hipertrófica/fisiopatologia , Transtornos do Crescimento/fisiopatologia , Síndrome de Noonan/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Adolescente , Adulto , Grupo com Ancestrais do Continente Asiático/genética , Estatura , Cardiomiopatia Hipertrófica/etiologia , Criança , Pré-Escolar , Feminino , Estudos de Associação Genética , Transtornos do Crescimento/etiologia , Humanos , Lactente , Japão , Masculino , Mutação , Síndrome de Noonan/complicações , Síndrome de Noonan/genética , Proteína Tirosina Fosfatase não Receptora Tipo 11/genética , Estenose da Valva Pulmonar/etiologia , Proteína SOS1/genética , Adulto Jovem
14.
Pediatr Cardiol ; 40(7): 1509-1515, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31342118

RESUMO

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.


Assuntos
Transposição Congênita Corrigida de Grandes Artérias/mortalidade , Comunicação Interventricular/mortalidade , Estenose da Valva Pulmonar/mortalidade , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Transposição Congênita Corrigida de Grandes Artérias/cirurgia , Feminino , Comunicação Interventricular/fisiopatologia , Humanos , Lactente , Masculino , Gravidez , Diagnóstico Pré-Natal , Estenose da Valva Pulmonar/fisiopatologia , Adulto Jovem
15.
Congenit Heart Dis ; 14(6): 1037-1045, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31250555

RESUMO

OBJECTIVE: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). BACKGROUND: Long-term data following BPV is limited to small, single center studies. METHODS: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. RESULTS: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. CONCLUSION: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.


Assuntos
Valvuloplastia com Balão/efeitos adversos , Hemodinâmica , Insuficiência da Valva Pulmonar/epidemiologia , Estenose da Valva Pulmonar/terapia , Valva Pulmonar/fisiopatologia , Adolescente , Adulto , Idoso , Peso Corporal , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , New York/epidemiologia , Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/fisiopatologia , Recuperação de Função Fisiológica , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
16.
JACC Cardiovasc Interv ; 12(16): 1594-1603, 2019 08 26.
Artigo em Inglês | MEDLINE | ID: mdl-31202951

RESUMO

OBJECTIVES: The aim of this study was to report long-term outcomes after percutaneous pulmonary valve implantation (PPVI). BACKGROUND: Excellent short- and mid-term results after PPVI for right ventricular outflow tract (RVOT) dysfunction have been reported. Data on long-term results after PPVI are scarce. METHODS: All 226 patients treated with PPVI at a single institution were prospectively enrolled in a database and included in the study. Follow-up information regarding outcomes was collected. Risk-factor analysis for death and reinterventions was performed. RESULTS: Follow-up data (up to 11 years) were available in 96% of the patients. At the end of the study period, 219 patients (92.8%) still lived with the initially implanted valve. Seven patients died, 2 of them from procedure-related complications. Seventeen patients (7.2%) needed valve replacement surgically (n = 11) or percutaneously (n = 6) for infectious endocarditis (n = 10) or valve degeneration (n = 7). In the remaining patients, the valves retained excellent function, and right ventricular dimensions and exercise capacity improved. A post-interventional RVOT gradient >15 mm Hg was a risk factor for death (hazard ratio: 7.57; 95% confidence interval: 1.26 to 45.38; p = 0.027) and for valve failure (hazard ratio: 3.76; 95% confidence interval: 1.43 to 9.93; p = 0.007). The best outcome was achieved with RVOT pre-stenting and a post-interventional gradient <15 mm Hg, resulting in an estimated event-free survival rate of 88% at 10 years. CONCLUSIONS: Patients after PPVI have excellent long-term outcomes. Right ventricular volumes, function, and exercise capacity improve significantly. A residual RVOT gradient <15 mm Hg was associated with the best outcome.


Assuntos
Cateterismo Cardíaco , Implante de Prótese de Valva Cardíaca , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Disfunção Ventricular Direita/cirurgia , Função Ventricular Direita , Pressão Ventricular , Adolescente , Adulto , Idoso , Valvuloplastia com Balão , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/mortalidade , Criança , Pré-Escolar , Bases de Dados Factuais , Remoção de Dispositivo , Feminino , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Estudos Prospectivos , Falha de Prótese , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/mortalidade , Estenose da Valva Pulmonar/fisiopatologia , Fatores de Risco , Fatores de Tempo , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/mortalidade , Disfunção Ventricular Direita/fisiopatologia , Adulto Jovem
17.
Am Heart J ; 213: 8-17, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31071505

RESUMO

BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ±â€¯6.4% vs. 48.5 ±â€¯7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ±â€¯17.7% vs. 75.6 ±â€¯20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ±â€¯3.9 vs.-25.7 ±â€¯4.4, P < .001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.


Assuntos
Tolerância ao Exercício/fisiologia , Hipertrofia Ventricular Direita/fisiopatologia , Insuficiência da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/fisiopatologia , Tetralogia de Fallot/fisiopatologia , Função Ventricular Direita/fisiologia , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia , Teste de Esforço , Feminino , Humanos , Hipertrofia Ventricular Direita/diagnóstico por imagem , Hipertrofia Ventricular Direita/etiologia , Hipertrofia Ventricular Direita/patologia , Imagem por Ressonância Magnética , Masculino , Análise por Pareamento , Consumo de Oxigênio , Complicações Pós-Operatórias/fisiopatologia , Atresia Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/complicações , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Volume Sistólico , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia
18.
Congenit Heart Dis ; 14(5): 691-699, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30989806

RESUMO

BACKGROUND: Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS). METHODS: A retrospective review was performed of all patients with TOF and pulmonary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early childhood (<8 years). Student's t test was performed to compare patients who underwent valve sparing (VS) versus transannular patch (TAP) repair. ANOVA was used to track measures of ventricular systolic function over time. RESULTS: We analyzed 151 echocardiograms performed on 42 patients. Pulmonary regurgitation (PR, moderate or severe) and the RV to left ventricular (LV) basal dimension ratio were higher in TAP patients (P < .04 at all-time points). Along with a significant increase in RV basal diameter Z-score in the TAP group (P < .001), there was an improvement in RV and LV GLS over time in both groups (P < .001). The LV GLS at last follow-up was lower in patients who underwent reoperation than those who did not (P = .050). LV GLS at the last follow-up echocardiogram was lower in patients with significant PR than those without (P < .001). CONCLUSIONS: Ventricular function appeared improve over time from the initial postoperative period in TOF patients. TAP repair was associated with a progressively higher RV/LV ratio in young children. GLS and RV/LV basal diameter ratio may be useful when following young children after TOF repair. Further research is necessary to understand the trajectory of ventricular functional and volumetric changes in young children in order to provide the most effective lifetime management of patients with TOF.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Ventrículos do Coração/fisiopatologia , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Tetralogia de Fallot/cirurgia , Função Ventricular Direita/fisiologia , Criança , Pré-Escolar , Ecocardiografia , Feminino , Seguimentos , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Masculino , Período Pós-Operatório , Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico , Estenose da Valva Pulmonar/fisiopatologia , Estudos Retrospectivos , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/fisiopatologia
19.
Int J Cardiovasc Imaging ; 35(8): 1453-1463, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30937683

RESUMO

To compare contrast-enhanced magnetic resonance angiography (ceMRA) and 3D steady-state free precession (SSFP) during systole and diastole for assessment of the right ventricle outflow tract (RVOT) in patients considered for percutaneous pulmonary valve implantation (PPVI) after tetralogy of Fallot (TOF) repair. We retrospectively evaluated 89 patients (male: 45, mean age 19 ± 8 years), who underwent cardiac-MRI after surgical TOF-repair. Datasets covering the whole heart in systole and diastole were acquired using ECG-gated 3D SSFP and non-gated ceMRA. Measurements were performed in SSFP-sequences and in ceMRA in the narrowest region of the RVOT to obtain the minimum, maximum and effective diameter. Invasive balloon sizing as the gold standard was available in 12 patients. The minimum diameter in diastolic SSFP, systolic SSFP and ceMRA were 21.4 mm (± 6.1 mm), 22.6 mm (± 6.2 mm) and 22.6 mm (± 6.0 mm), respectively. Maximum diameter was 29.9 mm (± 9.5 mm), 30.0 mm (± 7.0 mm) and 28.8 mm (± 8.1 mm) respectively. The effective diameter was 23.2 mm (± 5.7 mm), 27.4 mm (± 6.7 mm) and 24.4 mm (± 6.2 mm), differing significantly between diastole and systole (p < 0.0001). Measurements in ECG-gated SSFP showed a better inter- and intraobserver variability compared to measurements in non-ECG-gated ceMRA. Comparing invasive balloon sizing with our analysis, we found the highest correlation coefficients for the maximum and effective diameter measured in systolic SSFP (R = 0.99 respectively). ECG-gated 3D SSFP enables the identification and characterization of a potential landing zone for PPVI. The maximum and effective systolic diameter allow precise sizing for PPVI. Patients with TOF-repair could benefit from cardiac MRI before PPVI.


Assuntos
Cateterismo Cardíaco/métodos , Procedimentos Cirúrgicos Cardíacos , Implante de Prótese de Valva Cardíaca/métodos , Ventrículos do Coração/diagnóstico por imagem , Angiografia por Ressonância Magnética/métodos , Imagem por Ressonância Magnética/métodos , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Adolescente , Adulto , Valvuloplastia com Balão , Cateterismo Cardíaco/instrumentação , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Técnicas de Imagem de Sincronização Cardíaca , Criança , Meios de Contraste/administração & dosagem , Eletrocardiografia , Feminino , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/instrumentação , Ventrículos do Coração/fisiopatologia , Humanos , Interpretação de Imagem Assistida por Computador , Imageamento Tridimensional , Masculino , Variações Dependentes do Observador , Valor Preditivo dos Testes , Desenho de Prótese , Valva Pulmonar/fisiopatologia , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/etiologia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/cirurgia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/fisiopatologia , Resultado do Tratamento , Função Ventricular Direita , Adulto Jovem
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