RESUMO
BACKGROUND: There are limited data about the impact of timing of pulmonary valve replacement (PVR) on right heart reverse remodeling in patients with pulmonary regurgitation following intervention for isolated pulmonary valve stenosis (PS). This study compared differences in postprocedural right heart reverse remodeling after early versus late PVR (defined as PVR before versus after attainment of the conservative consensus criteria proposed by Bokma et al, 2018) in patients with prior intervention for PS, using patients with tetralogy of Fallot as the reference group. METHOD AND RESULTS: Right atrial reservoir strain and right ventricular free wall strain was measured at baseline, 1 and 3 years after PVR. There were 114 patients with PS (early PVR, 87 [76%]; late PVR, 27 [24%]) and 291 patients with tetralogy of Fallot (early PVR, 197 [67%]; late PVR, 96 [33%]). The PS group had greater improvement in right atrial reservoir strain at 1 year (12%±4% versus 8%±4%; P<0.001) and 3 years (15%±6% versus 9%±6%; P<0.001), and a greater improvement in right ventricular free wall strain at 1 year (12%±4% versus 7%±3%, P=0.008) and 3-years (16%±6% versus 12%±5%; P=0.01) after PVR compared with the tetralogy of Fallot group. There was no difference in right heart reverse remodeling between patients who underwent early versus later PVR within the PS group. In contrast, late PVR was associated with less right heart reverse remodeling within the tetralogy of Fallot group. CONCLUSIONS: These data suggest that patients with palliated PS presenting pulmonary regurgitation have a more benign clinical course, and hence delaying PVR in this population may be appropriate.
Assuntos
Implante de Prótese de Valva Cardíaca , Estenose da Valva Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Função Ventricular Direita , Remodelação Ventricular , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/fisiopatologia , Tetralogia de Fallot/complicações , Estenose da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/complicações , Masculino , Feminino , Função Ventricular Direita/fisiologia , Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Adulto , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/etiologia , Resultado do Tratamento , Adulto Jovem , Fatores de Tempo , Estudos Retrospectivos , AdolescenteAssuntos
Valvuloplastia com Balão , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Desenho de Prótese , Valva Pulmonar , Humanos , Resultado do Tratamento , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/efeitos adversos , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Masculino , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/fisiopatologia , Feminino , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/etiologiaRESUMO
BACKGROUND: Studies of transcatheter pulmonary valve replacement (TPVR) with the Melody valve have demonstrated good clinical and hemodynamic outcomes. Our study analyzes the midterm clinical and hemodynamic outcomes for patients who underwent Melody valve implantation in Southeast Asia. METHODS: Patients with circumferential conduits or bioprosthetic valves and experiencing post-operative right ventricular outflow tract (RVOT) dysfunction were recruited for Melody TPVR. RESULTS: Our cohort (n = 14) was evenly divided between pediatric and adult patients. The median age was 19 years (8-38 years), a male-to-female ratio of 6:1 with a median follow-up period of 48 months (16-79 months), and the smallest patient was an 8-year-old boy weighing 18 kg. All TPVR procedures were uneventful and successful with no immediate mortality or conduit rupture. The primary implant indication was combined stenosis and regurgitation. The average conduit diameter was 21 ± 2.3 mm. Concomitant pre-stenting was done in 71.4% of the patients without Melody valve stent fractures (MSFs). Implanted valve size included 22-mm (64.3%), 20-mm (14.3%), and 18-mm (21.4%). After TPVR, the mean gradient across the RVOT was significantly reduced from 41 mmHg (10-48 mmHg) to 16 mmHg (6-35 mmHg) at discharge, p < 0.01. Late follow-up infective endocarditis (IE) was diagnosed in 2 patients (14.3%). Overall freedom from IE was 86% at 79 months follow-up. Three patients (21.4%) developed progressive RVOT gradients. CONCLUSION: For patients in Southeast Asia with RVOT dysfunction, Melody TPVR outcomes are similar to those reported for patients in the US in terms of hemodynamic and clinical improvements. A pre-stenting strategy was adopted and no MSFs were observed. Post-implantation residual stenosis and progressive stenosis of the RVOT require long term monitoring and reintervention. Lastly, IE remained a concern despite vigorous prevention and peri-procedural bacterial endocarditis prophylaxis.
Assuntos
Cateterismo Cardíaco , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Hemodinâmica , Desenho de Prótese , Valva Pulmonar , Recuperação de Função Fisiológica , Humanos , Masculino , Criança , Feminino , Implante de Prótese de Valva Cardíaca/instrumentação , Implante de Prótese de Valva Cardíaca/efeitos adversos , Adolescente , Valva Pulmonar/cirurgia , Valva Pulmonar/fisiopatologia , Valva Pulmonar/diagnóstico por imagem , Resultado do Tratamento , Adulto Jovem , Cateterismo Cardíaco/instrumentação , Cateterismo Cardíaco/efeitos adversos , Fatores de Tempo , Adulto , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/fisiopatologia , Insuficiência da Valva Pulmonar/etiologia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Bioprótese , Estenose da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Sudeste AsiáticoRESUMO
d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3-5% of all congenital heart defects. In a simple d-TGA (about two-thirds of patients), there is no other cardiac abnormality other than a patent foramen ovale (PFO) and a patent ductus arteriosus (PDA). In a complex d-TGA additional cardiac abnormalities such as VSD, pulmonary stenosis or coronary abnormalities are present. About one-third to 40% of patients with d-TGA have an associated ventricular septal defect. Among patients with d-TGA, 6% of those with intact ventricular septum and 31% of those with ventricular septal defect have associated pulmonary stenosis. Coronary abnormalities are of importance with regard to the complexity of surgical repair.
Assuntos
Transposição dos Grandes Vasos , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/terapia , Humanos , Recém-Nascido , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/terapia , Procedimentos Cirúrgicos Cardíacos/métodos , Estenose da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/terapia , Estenose da Valva Pulmonar/diagnóstico por imagemRESUMO
Lesions of the semilunar valve and the aortic arch can occur either in isolation or as part of well-described clinical syndromes. The polygenic cause of calcific aortic valve disease will be discussed including the key role of NOTCH1 mutations. In addition, the complex trait of bicuspid aortic valve disease will be outlined, both in sporadic/familial cases and in the context of associated syndromes, such as Alagille, Williams, and Kabuki syndromes. Aortic arch abnormalities particularly coarctation of the aorta and interrupted aortic arch, including their association with syndromes such as Turner and 22q11 deletion, respectively, are also discussed. Finally, the genetic basis of congenital pulmonary valve stenosis is summarized, with particular note to Ras-/mitogen-activated protein kinase (Ras/MAPK) pathway syndromes and other less common associations, such as Holt-Oram syndrome.
Assuntos
Aorta Torácica , Valva Aórtica , Humanos , Aorta Torácica/anormalidades , Aorta Torácica/patologia , Valva Aórtica/anormalidades , Valva Aórtica/patologia , Anormalidades Múltiplas/genética , Anormalidades Múltiplas/patologia , Cardiopatias Congênitas/genética , Cardiopatias Congênitas/patologia , Doença da Válvula Aórtica Bicúspide/genética , Estenose da Valva Pulmonar/genética , Mutação , Receptor Notch1/genética , Valvopatia Aórtica/genética , Doenças das Valvas Cardíacas/genética , Doenças das Valvas Cardíacas/patologia , Calcinose/genética , Calcinose/patologia , Doenças Hematológicas/genética , Doenças Hematológicas/patologia , Doenças Vestibulares/genética , Doenças Vestibulares/patologiaRESUMO
BACKGROUND: The survival of patients with neuroendocrine tumors has substantially improved with modern treatment options. Although the associated carcinoid syndrome can be diagnosed early and controlled effectively, cardiologists still encounter patients with cardiac manifestations, particularly among individuals with persistently high levels of vasoactive mediators. Treatment options have been limited to surgical valve replacement in fully manifested disease. Since surgery is not always feasible, transcatheter valve implantation is becoming an interesting alternative. CASE: A case of a 50-year-old woman with carcinoid syndrome and right-sided valvular heart disease is presented. Moderate pulmonary valve stenosis and severe tricuspid valve regurgitation were diagnosed during the evaluation and treatment of neuroendocrine tumor. The possibility of rare valve involvement and the need for interdisciplinary cooperation in the diagnosis, monitoring and treatment of patients with neuroendocrine tumors producing vasoactive substances must be emphasized. CONCLUSION: The patient had a typically presenting carcinoid syndrome with a rare cardiac manifestation. Although monitoring and treatment were carried out in accordance with recommendations and appropriate to the clinical condition, rapid progression of the metastatic disease ultimately precluded invasive cardiac intervention.
Assuntos
Doença Cardíaca Carcinoide , Insuficiência da Valva Tricúspide , Humanos , Feminino , Pessoa de Meia-Idade , Doença Cardíaca Carcinoide/diagnóstico , Insuficiência da Valva Tricúspide/etiologia , Estenose da Valva Pulmonar , Síndrome do Carcinoide MalignoRESUMO
Transcatheter pulmonary valve implantation (TPVI) is indicated for use in the management of failing pulmonary valves in humans. We report here the long-term follow-up of the first documented transcatheter pulmonary valve implanted in a client-owned dog. A one-year-old Beagle dog with severe congenital type A valvular pulmonic stenosis first underwent percutaneous balloon pulmonary valvuloplasty, leading two years later to severe pulmonary regurgitation. A TPVI using a Melody™ bioprosthetic valve was then successfully performed, with normalization of the right heart cavities. Repeated two- and three-dimensional transthoracic echocardiographic examinations combined with Doppler modes confirmed the appropriate position and function of the valve for four years. Mitral myxomatous valvular degeneration led to refractory left-sided congestive heart failure, and the dog was humanely euthanized. After postmortem examination, X-ray imaging and histopathological evaluation of the stent and the valve were performed. Ex-vivo imaging of the implanted valve using a Faxitron® Path radiography system and microscopic evaluation of the implanted stent and bioprosthetic leaflets did not show any relevant leaflet or stent alterations. This case provides a proof of concept in interventional veterinary cardiology, showing that TPVI can be performed in dogs with subsequent long-term maintaining normal pulmonary valve function.
Assuntos
Doenças do Cão , Implante de Prótese de Valva Cardíaca , Estenose da Valva Pulmonar , Valva Pulmonar , Animais , Cães , Doenças do Cão/cirurgia , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/patologia , Estenose da Valva Pulmonar/veterinária , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Valva Pulmonar/cirurgia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/patologia , Implante de Prótese de Valva Cardíaca/veterinária , Implante de Prótese de Valva Cardíaca/instrumentação , Ecocardiografia Tridimensional/veterinária , Insuficiência da Valva Pulmonar/veterinária , Insuficiência da Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Ecocardiografia/veterinária , Bioprótese/veterinária , Masculino , Próteses Valvulares Cardíacas/veterinária , FemininoRESUMO
BACKGROUND: Peripheral pulmonary stenosis (PPS) is a condition characterized by the narrowing of the pulmonary arteries, which impairs blood flow to the lung. The mechanisms underlying PPS pathogenesis remain unclear. Thus, the aim of this study was to investigate the genetic background of patients with severe PPS to elucidate the pathogenesis of this condition. METHODS AND RESULTS: We performed genetic testing and functional analyses on a pediatric patient with PPS and Williams syndrome (WS), followed by genetic testing on 12 patients with WS and mild-to-severe PPS, 50 patients with WS but not PPS, and 21 patients with severe PPS but not WS. Whole-exome sequencing identified a rare PTGIS nonsense variant (p.E314X) in a patient with WS and severe PPS. Prostaglandin I2 synthase (PTGIS) expression was significantly downregulated and cell proliferation and migration rates were significantly increased in cells transfected with the PTGIS p.E314X variant-encoding construct when compared with that in cells transfected with the wild-type PTGIS-encoding construct. p.E314X reduced the tube formation ability in human pulmonary artery endothelial cells and caspase 3/7 activity in both human pulmonary artery endothelial cells and human pulmonary artery smooth muscle cells. Compared with healthy controls, patients with PPS exhibited downregulated pulmonary artery endothelial prostaglandin I2 synthase levels and urinary prostaglandin I metabolite levels. We identified another PTGIS rare splice-site variant (c.1358+2T>C) in another pediatric patient with WS and severe PPS. CONCLUSIONS: In total, 2 rare nonsense/splice-site PTGIS variants were identified in 2 pediatric patients with WS and severe PPS. PTGIS variants may be involved in PPS pathogenesis, and PTGIS represents an effective therapeutic target.
Assuntos
Sistema Enzimático do Citocromo P-450 , Oxirredutases Intramoleculares , Estenose da Valva Pulmonar , Síndrome de Williams , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Movimento Celular , Proliferação de Células , Células Cultivadas , Códon sem Sentido , Células Endoteliais/enzimologia , Células Endoteliais/metabolismo , Sequenciamento do Exoma , Predisposição Genética para Doença , Oxirredutases Intramoleculares/genética , Oxirredutases Intramoleculares/metabolismo , Fenótipo , Artéria Pulmonar/fisiopatologia , Artéria Pulmonar/enzimologia , Estenose da Valva Pulmonar/genética , Estenose da Valva Pulmonar/fisiopatologia , Índice de Gravidade de Doença , Síndrome de Williams/genética , Síndrome de Williams/fisiopatologia , Síndrome de Williams/enzimologiaRESUMO
BACKGROUND Congenital heart diseases (CHDs) are the most common form of birth defects, affecting the structure and function of neonatal hearts. Pulmonary valve stenosis (PVS) and ventricular septal defects (VSD) are 2 of the more prevalent forms, both of which can lead to significant morbidity if left untreated. The emergence of transcatheter techniques has revolutionized the therapeutic landscape, presenting minimally invasive yet effective alternatives to open-heart surgery and significantly reducing associated patient morbidity and recovery time. CASE REPORT The presented case details the management of a 19-year-old man with complex CHDs, highlighting the nuanced decision-making process that led to a transcatheter approach. The patient's clinical presentation, marked by symptoms reflective of significant cardiac compromise, demanded a tailored approach that utilized the latest advancements in non-surgical intervention. The successful closure of the VSD with an Amplatzer device and the resolution of PVS via balloon valvuloplasty were achieved without complications, showcasing the potential of these techniques in managing similar cases. The post-intervention period was marked by a noteworthy recovery, confirming the procedural efficacy and enhancing the patient's quality of life. CONCLUSIONS The favorable outcome of this case highlights the pivotal role of transcatheter interventions in treating complex CHDs and suggests a shift towards less invasive approaches in cardiac care. This case contributes valuable insights to the existing body of evidence, reinforcing the potential of transcatheter techniques to become the preferred treatment modality. With promising immediate and short-term results, these techniques highlight the need for continued research into their long-term efficacy and application across diverse patient demographics.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Comunicação Interventricular , Estenose da Valva Pulmonar , Humanos , Masculino , Adulto Jovem , Cateterismo Cardíaco/métodos , Cardiopatias Congênitas/complicações , Comunicação Interventricular/cirurgia , Estenose da Valva Pulmonar/cirurgia , Qualidade de Vida , Resultado do TratamentoRESUMO
Paciente masculino de 45 años con antecedente de estenosis pulmonar diagnosticada por ecocardiografía. Se efectuó un Angiotac coronario para definir mejor la anatomía y planificar la instalación de una válvula percutánea. El Angiotac coronario mostró una válvula pulmonar cuadricúspide, con leve engrosamiento de los velos, que presentan poca movilidad durante el ciclo cardíaco, lo que determina estenosis e insuficiencia. El área de estenosis por planimetría era 1.1 cm2, y el área de insuficiencia 1.2 cm2. Además, se observó aumento de calibre de la arteria pulmonar, hipertrofia ventricular derecha y aumento de tamaño de la aurícula derecha.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Valva Pulmonar/anormalidades , Valva Pulmonar/diagnóstico por imagem , Angiografia por Tomografia Computadorizada/métodos , Estenose da Valva PulmonarRESUMO
BACKGROUND: Congenital heart disease poses a therapeutic challenge, specifically pulmonary valve stenosis. This has been treated for many years with invasive procedures and bioprostheses, which over time, become dysfunctional due to the accumulation of fibrous tissue and calcification. OBJECTIVE: The aim of this study is to describe the use of endovascular management in the right ventricular outflow tract, as the beginning of an ongoing effot to improve pediatric outcomes in developing countries. METHODS: Seven pediatric patients with endovascular management of the right outflow tract are presented. Three of them underwent surgical valvuloplasty with persistent pulmonary stenosis. They decided to insert a percutaneous transcatheter pulmonary valve (PPVI) with a Melody valve using the valve-in-valve technique, with 100% stenosis and no complications associated with the procedure. RESULTS: Four patients with successful percutaneous valve implantation had different congenital heart diseases. In addition, the case of a patient in whom an intentional pulmonary valve fracture was performed, an innovative procedure in pediatric endovascular management in the country, is highlighted. CONCLUSIONS: The procedure was minimally invasive, safe, and effective. The IVPP technique could be a viable option in our country for managing failed primary valve implantations or even in native tracts.
ANTECEDENTES: Las cardiopatías congénitas plantean un desafío terapéutico, específicamente la estenosis de la válvula pulmonar. Esta ha sido tratada durante muchos años con procedimientos invasivos e inserción de bioprótesis, que con el tiempo se vuelven disfuncionales y pueden reestenosarse por acumulación de tejido fibroso y calcificación. Debido a las complicaciones generadas por la injuria quirúrgica, se han descrito medidas menos invasivas para el manejo de la estenosis residual e inicial por medios endovasculares en adultos y más recientemente en población pediátrica. OBJETIVO: El objetivo de este reporte es describir la misma en el manejo endovascular del tracto de salida del ventrículo derecho, como el inicio de un trabajo continuo para la mejoría de los resultados pediátricos en países en vía de desarrollo. MÉTODOS: Se presentan siete casos pediátricos de manejo endovascular del tracto de salida derecho; tres de ellos sometidos a valvuloplastia quirúrgica con persistencia de la estenosis pulmonar, por lo cual se decidió inserción percutánea de una válvula pulmonar (IVPP) transcatéter con válvula Melody utilizando la técnica valve-in-valve, con lo que se consiguió una resolución del 100% de la estenosis y no se presentó ningún tipo de complicación asociada al procedimiento. RESULTADOS: En cuatro pacientes se logró una implantación exitosa de la válvula por vía percutánea en diferentes cardiopatías congénitas, siendo uno de ellos en tracto nativo; además, destaca el caso de un paciente en quien se realizó fractura intencional de la válvula pulmonar, procedimiento innovador en el manejo endovascular pediátrico en Colombia. CONCLUSIONES: En estos pacientes el procedimiento resultó ser poco invasivo, seguro y efectivo. La técnica IVPP podría ser considerada una opción viable en Colombia (y en otros países en desarrollo) para el manejo de implantes valvulares primarios fallidos o incluso en tractos nativos.
Assuntos
Cardiopatias Congênitas , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Estenose da Valva Pulmonar , Valva Pulmonar , Humanos , Criança , Implante de Prótese de Valva Cardíaca/métodos , Colômbia , Resultado do Tratamento , Cateterismo Cardíaco/métodos , Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/cirurgia , Cardiopatias Congênitas/cirurgia , Desenho de PróteseRESUMO
The aortic short axis view demonstrated the widening of the pulmonary artery and the membrane-like echo in the pulmonary artery divided it into true lumen and false lumen. And the flow of the ruptured openings on the band-like echo was clearly revealed by Color Doppler.
Assuntos
Dissecção Aórtica , Comunicação Interventricular , Estenose da Valva Pulmonar , Humanos , Ecocardiografia , Artéria Pulmonar/diagnóstico por imagem , Comunicação Interventricular/complicações , Comunicação Interventricular/diagnóstico por imagem , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/diagnóstico por imagemRESUMO
RATIONALE: Summarizing the perioperative nursing experience in the successful treatment of 4 neonates with critical pulmonary stenosis (CPS). PATIENT CONCERNS: Of the 4 patients, 3 had postnatal shortness of breath and varying degrees of cyanosis, aggravated by crying and noise, and 1 had no obvious shortness of breath and cyanosis. The preoperative auscultation of the precordial region could be heard 3-4/6 systolic murmur; echocardiography was diagnosed as CPS, combined with patent ductus arteriosus, right ventricular dysplasia, and severe tricuspid regurgitation. Four children were treated with prostaglandin 5 ng/(kg-min) to maintain a certain degree of pulmonary blood flow to improve hypoxemia, effectively preventing ductus arteriosus from closure, and the infusion was discontinued 2 hours prior to the operation. Three of the children required ventilator-assisted respiration to relieve severe hypoxia and correct acidosis before surgery. DIAGNOSIS: Neonatal CPS was diagnosed. INTERVENTIONS: Four neonates with rapidly developing conditions were admitted to the hospital, a multidisciplinary in-hospital consultation was organized immediately, and a multidisciplinary collaborative team was set up, consisting of medical doctors and nurses from the medical department, the neonatal intensive care unit, cardiovascular medicine, cardiac ultrasound room, anesthesiology department, and radiology and interventional medicine department. The multidisciplinary team evaluated the treatment modality of the children and finally decided to perform percutaneous balloon pulmonary valvuloplasty. The surgical team included specialists from the Department of Cardiovascular Medicine, Department of Interventional Radiology, Cardiac Ultrasound Unit, and Department of Anesthesiology. OUTCOMES: All 4 neonates were successfully operated and discharged from the hospital. Multidisciplinary follow-up interventions were carried out 1 year after discharge, and the children were in good condition. LESSONS: The specialty nursing-led multidisciplinary collaboration model significantly improves the professional competence of nurses from various specialties, promotes the integration and development of multispecialty disciplines, and provides better quality services for children, which is the key to improving the success rate of percutaneous balloon pulmonary valvuloplasty in neonates.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Permeabilidade do Canal Arterial , Estenose da Valva Pulmonar , Recém-Nascido , Criança , Humanos , Estenose da Valva Pulmonar/cirurgia , Assistência Perioperatória , Cianose , DispneiaRESUMO
BACKGROUND: Brachycephalic dogs with pulmonary stenosis are known to have a higher incidence of concurrent coronary artery abnormalities than non-brachycephalic breeds, which increases risk when performing balloon valvuloplasty. The use of ECG-gated CT angiography has been reported for the evaluation of coronary arteries in normal dogs and dogs with pulmonary stenosis. The purpose of this study was to report findings of coronary artery origination and morphology of main branches using ECG-gated CT angiography in brachycephalic dogs with pulmonary stenosis. METHODS: An ECG-gated CT angiographic protocol was used to image coronary artery anatomy in nine brachycephalic dogs with pulmonary stenosis. Images were assessed for quality as well as coronary artery morphology by one veterinary radiologist, one veterinary cardiologist and one veterinary radiology resident. RESULTS: All nine dogs had good to excellent image quality. Coronary artery anomalies were identified in three of nine dogs: one R2A anomaly, one L2A anomaly and one L2C anomaly. Two dogs were assessed to be poor balloon valvuloplasty candidates based on CT angiographic images. LIMITATION: Coronary artery morphology was not confirmed via postmortem examination in all patients. CONCLUSION: ECG-gated CT angiography is a minimally invasive imaging modality capable of diagnosing various coronary artery anomalies in brachycephalic dogs with pulmonary stenosis and aiding in the determination of patient candidacy for balloon valvuloplasty.
Assuntos
Craniossinostoses , Doenças do Cão , Cardiopatias Congênitas , Estenose da Valva Pulmonar , Cães , Animais , Angiografia por Tomografia Computadorizada/veterinária , Vasos Coronários , Angiografia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/veterinária , Cardiopatias Congênitas/veterinária , Craniossinostoses/veterinária , Eletrocardiografia/veterinária , Doenças do Cão/diagnóstico por imagemRESUMO
BACKGROUND: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment. CASE PRESENTATION: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful. CONCLUSION: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Estenose da Valva Pulmonar , Valva Pulmonar , Transposição dos Grandes Vasos , Humanos , Masculino , Pré-Escolar , Adulto Jovem , Adulto , Transposição das Grandes Artérias Corrigida Congenitamente , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/cirurgia , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/complicações , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Resultado do TratamentoRESUMO
Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.
Assuntos
Valvuloplastia com Balão , Permeabilidade do Canal Arterial , Canal Arterial , Estenose da Valva Pulmonar , Lactente , Feminino , Gravidez , Humanos , Recém-Nascido , Estudos Retrospectivos , Ecocardiografia , Terceiro Trimestre da Gravidez , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Ultrassonografia Pré-NatalRESUMO
This study describes right ventricle (RV) characteristics and right ventricle to pulmonary artery (RV-PA) conduit function pre- and post-repair in patients with tetraology of Fallot with major aortopulmonary collaterals (TOF/MAPCAs). We reviewed patients who underwent single-stage, complete unifocalization, and repair of TOF/MAPCAs between 2006 and 2019 with available pre- and early postoperative echocardiograms. For a subset of patients, 6-12 month follow-up echocardiogram was available. RV and left ventricle (LV) characteristics and RV-PA conduit function were reviewed. Wilcoxon signed rank test and McNemar's test were used. 170 patients were reviewed, 46 had follow-up echocardiograms. Tricuspid valve annular plane systolic excursion (TAPSE) Z-scores were reduced from pre- (Z-score 0.01) to post-repair (Z-score -4.5, p < 0.001), improved but remained abnormal at follow-up (Z-score -4.0, p < 0.001). RV fractional area change (FAC) and LV ejection fraction were not significantly different before and after surgery. Conduit regurgitation was moderate or greater in 11% at discharge, increased to 65% at follow-up. RV-PA conduit failure (severe pulmonary stenosis or severe pulmonary regurgitation) was noted in 61, and 63% had dilated RV (diastolic RV area Z-score > 2) at follow-up. RV dilation correlated with the severe conduit regurgitation (p = 0.018). Longitudinal RV function was reduced after complete repair of TOF/MAPCAs, with decreased TAPSE and preserved FAC and LV ejection fraction. TAPSE improved but did not normalize at follow-up. Severe RV-PA conduit dysfunction was observed prior to discharge in 11% of patients and in 61% at follow-up. RV dilation was common at follow-up, especially in the presence of severe conduit regurgitation.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Insuficiência Cardíaca , Estenose da Valva Pulmonar , Tetralogia de Fallot , Humanos , Ventrículos do Coração , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Estudos Retrospectivos , Estenose da Valva Pulmonar/cirurgia , Função Ventricular DireitaRESUMO
Pulmonary homograft dysfunction is challenging to treat in patients with a previous Ross procedure, and results in significant morbidity and mortality in case of reoperation. We report the case of a patient with early severe pulmonary homograft stenosis 18 months after a Ross procedure and successful management using transcatheter pulmonary valve replacement.
Assuntos
Implante de Prótese de Valva Cardíaca , Estenose da Valva Pulmonar , Valva Pulmonar , Humanos , Valva Pulmonar/cirurgia , Valva Pulmonar/transplante , Resultado do Tratamento , Transplante Homólogo , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Reoperação , Aloenxertos/cirurgia , Valva Aórtica/cirurgia , SeguimentosRESUMO
BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.