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1.
Am J Forensic Med Pathol ; 41(1): 40-41, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31929320

RESUMO

The deceased was a 44-year-old male who was treated for a suspected Ebstein's anomaly observed using transthoracic echocardiogram. He was found dead in his bed at home. Autopsy revealed that the septal tricuspid leaflet was intact; however, a large anterior tricuspid leaflet cleft and right atrioventricular cavity dilation were observed. Pathological examination revealed a normal tricuspid valve, except for the presence of a cleft with local fibrosis of the left ventricle papillary muscle and hemosiderin-containing macrophages at both lungs. There were no other abnormalities that may have led to death. It was concluded that he died a cardiac death based on the right heart overload associated with the anterior tricuspid leaflet cleft. This case indicates the possibility that the anterior tricuspid leaflet cleft can cause death and also highlights the necessity of a detailed autopsy to accurately diagnose the cause of death.


Assuntos
Valva Tricúspide/anormalidades , Valva Tricúspide/patologia , Adulto , Proteína C-Reativa/análise , Diagnóstico Diferencial , Anomalia de Ebstein/diagnóstico , Fibrose , Patologia Legal , Insuficiência Cardíaca/etiologia , Ventrículos do Coração/patologia , Hemossiderina/metabolismo , Humanos , Pulmão/metabolismo , Macrófagos/metabolismo , Masculino , Peptídeo Natriurético Encefálico/sangue , Músculos Papilares/patologia , Fragmentos de Peptídeos/sangue , Insuficiência da Valva Tricúspide/complicações
3.
Methodist Debakey Cardiovasc J ; 15(2): 138-144, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384377

RESUMO

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Fármacos Cardiovasculares/uso terapêutico , Anomalia de Ebstein/terapia , Insuficiência da Valva Tricúspide/terapia , Valva Tricúspide/efeitos dos fármacos , Valva Tricúspide/cirurgia , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Fármacos Cardiovasculares/efeitos adversos , Anomalia de Ebstein/complicações , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Hemodinâmica/efeitos dos fármacos , Humanos , Recuperação de Função Fisiológica , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/tratamento farmacológico , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/fisiopatologia , Função Ventricular Direita/efeitos dos fármacos
4.
Ann Thorac Cardiovasc Surg ; 25(5): 260-264, 2019 Oct 20.
Artigo em Inglês | MEDLINE | ID: mdl-31308306

RESUMO

PURPOSE: Different surgical procedures were defined due to degree of Ebstein anomaly. In this study, we are reporting our surgical experience of adult patients with Ebstein anomaly. METHODS: We analyzed the patients, who had operated with the diagnosis of Ebstein anomaly from March 2011 through February 2018, retrospectively. We evaluated patients in two groups: patients with cone type surgical repair were in Group 1 and patients with other surgical (Danielson, Carpentier) procedures were in Group 2. RESULTS: There were 23 patients in the study; 9 (39%) were in Group 1 and 14 (61%) were in Group 2. In the comparison of preoperative data, groups were statistically similar. Aortic cross clamp and cardiopulmonary bypass (CPB) times, need for inotropic agents, intubation time, postoperative >2 tricuspid regurgitation incidence, intensive care unit, and hospital staying times were statistically significantly higher in Group 2. There was one mortality (4.3%) in Group 2 due to sepsis. CONCLUSION: Cone type repair can safely be performed in the repair of Ebstein anomaly with acceptable results. Our results are encouraging to prefer this technique as one of the first-line treatment of Ebstein anomaly. However, further randomized controlled studies are recommended to evaluate the efficacy of this surgical procedure.


Assuntos
Anuloplastia da Valva Cardíaca , Anomalia de Ebstein/cirurgia , Implante de Prótese de Valva Cardíaca , Valva Tricúspide/cirurgia , Adulto , Anuloplastia da Valva Cardíaca/efeitos adversos , Anuloplastia da Valva Cardíaca/instrumentação , Anomalia de Ebstein/diagnóstico por imagem , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/instrumentação , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Adulto Jovem
5.
J Vet Cardiol ; 23: 129-141, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31174723

RESUMO

OBJECTIVES: The objective of this study was to report the signalment, presentation, clinical and imaging findings, interventions, and outcomes in a group of dogs with cor triatriatum dexter (CTD). ANIMALS: Seventeen client-owned dogs. METHODS: Medical records were reviewed retrospectively for signalment, history, physical examination findings, imaging and diagnostic findings, presence of concurrent congenital cardiac defects, description of interventional procedures, therapy information, and outcomes. RESULTS: Age at presentation ranged from two to 110 months, with 10 of 17 dogs (59%) aged <12 months. There was an equal distribution between the sexes. Peritoneal effusion was the most common presenting complaint, in 10 of 17 dogs (59%). The CTD was an isolated finding in 3 of 17 dogs (18%); the remaining 14 of 17 (82%) dogs had concurrent cardiac disease, with congenital anomalies present in 12 of 17 (70%). All except one of these 12 dogs had at least one additional condition affecting the right heart. Tricuspid valve dysplasia was the most common congenital comorbidity, present in 9 of 17 dogs (53%). Seven dogs (41%) underwent interventional treatment of their CTD. In 7 of 17 (41%) cases, the CTD was considered to be incidental and the dogs were asymptomatic; therefore, no interventions were performed. The remaining three cases were euthanized or lost to follow-up. CONCLUSIONS: Cor triatriatum dexter in dogs is commonly seen in association with other right-sided congenital cardiac anomalies and may be an incidental finding. Dogs with CTD obstructing right atrial inflow can have a good outcome after intervention. Dogs with no clinical signs associated with the CTD may remain asymptomatic into adulthood.


Assuntos
Coração Triatriado/veterinária , Doenças do Cão/diagnóstico , Doenças do Cão/terapia , Animais , Líquido Ascítico , Coração Triatriado/diagnóstico , Coração Triatriado/terapia , Cães , Feminino , Cardiopatias Congênitas/veterinária , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Valva Tricúspide/anormalidades
6.
J Cardiovasc Magn Reson ; 21(1): 34, 2019 06 27.
Artigo em Inglês | MEDLINE | ID: mdl-31242903

RESUMO

BACKGROUND: Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. METHODS: A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. RESULTS: Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14-57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60o, relative risk of dehiscence was 3.2 (CI 1.3-4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3-100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. CONCLUSIONS: Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/cirurgia , Imagem Cinética por Ressonância Magnética , Procedimentos Cirúrgicos Reconstrutivos , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Tomada de Decisão Clínica , Anomalia de Ebstein/mortalidade , Anomalia de Ebstein/fisiopatologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/mortalidade , Valor Preditivo dos Testes , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Procedimentos Cirúrgicos Reconstrutivos/mortalidade , Recuperação de Função Fisiológica , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Adulto Jovem
8.
JACC Cardiovasc Imaging ; 12(4): 637-651, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30947906

RESUMO

Tricuspid valve abnormalities in congenital heart disease comprise a wide spectrum, with the most common being Ebstein anomaly and tricuspid valve dysplasia. Tricuspid valve dysfunction may also be secondary to other types of congenital heart disease, including functional tricuspid regurgitation seen in right heart volume overload conditions, such as atrial septal defect and repaired tetralogy of Fallot with severe pulmonary valve regurgitation. Congenitally corrected transposition and Mustard and Senning procedures maintain the right ventricle as the systemic ventricle, and the tricuspid valve is subject to unique hemodynamic stress not typically seen in normal circulation. Surgical treatment of tricuspid valve disease remains the mainstay of therapy; primary catheter-based interventions are uncommon. However, once a tricuspid valve has been replaced with a bioprosthesis, the patient may be a candidate for tricuspid valve-in-valve catheter-based replacement.


Assuntos
Ecocardiografia , Cardiopatias Congênitas/diagnóstico por imagem , Imagem por Ressonância Magnética , Valva Tricúspide/diagnóstico por imagem , Fatores Etários , Bioprótese , Cateterismo Cardíaco/instrumentação , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Próteses Valvulares Cardíacas , Implante de Prótese de Valva Cardíaca/instrumentação , Humanos , Valor Preditivo dos Testes , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/fisiopatologia , Valva Tricúspide/cirurgia
9.
Artigo em Inglês | MEDLINE | ID: mdl-31027561

RESUMO

PA/IVS is a rare, heterogenous congenital heart defect anatomically defined by complete obstruction to the right ventricular outflow tract with varying degrees of hypoplasia of the right ventricle and tricuspid valve. This lesion can have associated coronary artery anomalies and, in some cases, right ventricular-dependent coronary circulation. Due to the wide spectrum of presenting anatomic and clinical features, the treatment options are often dictated by the degree of development of the tricuspid valve and right ventricle. The purpose of this review is to discuss the spectrum of pulmonary atresia with intact ventricular septum morphologies and to evaluate the surgical decision-making process and approaches to surgical repair with respect to the impact of hypoplastic right-sided cardiac features.


Assuntos
Tomada de Decisão Clínica , Cardiopatias Congênitas/cirurgia , Seleção de Pacientes , Atresia Pulmonar/cirurgia , Valva Tricúspide/anormalidades , Humanos , Recém-Nascido
10.
Cardiol Young ; 29(3): 451-453, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30714559

RESUMO

A newborn with prenatally diagnosed dysplasia of both atrioventricular valves presented after birth with signs and symptoms of low cardiac output, severe regurgitation of both mitral and tricuspid valves. This combination is as rare as challenging, since it regards both the timing and management of this complex cardiac malformation. We report an early surgical repair of both atrioventricular valves in a symptomatic newborn, which improved his clinical status and, so far, delayed valve replacement.


Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Doenças das Valvas Cardíacas/congênito , Valva Mitral/anormalidades , Valva Tricúspide/anormalidades , Ecocardiografia , Doenças das Valvas Cardíacas/diagnóstico , Doenças das Valvas Cardíacas/cirurgia , Humanos , Recém-Nascido , Masculino , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia
11.
Echocardiography ; 36(3): 615-617, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30738004

RESUMO

Unguarded tricuspid orifice is the most extreme of tricuspid valve dysplasia with a very variable natural history. They can tolerate tricuspid regurgitation well, and they become symptomatic only if significant right ventricular dysfunction or atrial fibrillation occurs. Patients with a mild degree of right ventricular dysfunction can survive to adulthood and even reach old age. Surgical treatment is a difficult option due to variable natural history, and surgical results are not too encouraging.


Assuntos
Ecocardiografia , Achados Incidentais , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Adulto , Anticoagulantes/uso terapêutico , Diuréticos/uso terapêutico , Ecocardiografia Tridimensional , Humanos , Masculino
12.
Semin Thorac Cardiovasc Surg ; 31(3): 566-568, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30735711

RESUMO

We describe a change in our technique for plication of the atrialized right ventricle during a Cone repair. The plication was performed from the "Outside"-on a beating heart. Advantages expected from this change are-all major coronaries are better seen when full of blood and can be avoided, if a major vessel is compromised-electrocardiography changes should point it out immediately, there is a small but significant saving in terms of arrest time and if the plication needs to be taken down because of an adverse coronary event-this can be done without the need of another cardioplegia.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Anomalia de Ebstein/cirurgia , Átrios do Coração/cirurgia , Ventrículos do Coração/cirurgia , Valva Tricúspide/cirurgia , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia
15.
Catheter Cardiovasc Interv ; 93(1): E46-E48, 2019 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-30280471

RESUMO

We report the use of an Amplatzer Vascular plug for percutaneous closure of a paravalvular leak after Cone repair in a 5-year-old boy with Ebstein's anomaly. A paravalvular leak of the tricuspid valve developed gradually after Cone repair-surgery. The combination of fluoroscopy, transesophageal and transthoracic imaging during general anesthesia was necessary for correct and safe positioning of the device by percutaneous approach. The results were promising.


Assuntos
Cateterismo Cardíaco , Anuloplastia da Valva Cardíaca/efeitos adversos , Anomalia de Ebstein/cirurgia , Insuficiência da Valva Tricúspide/cirurgia , Valva Tricúspide/cirurgia , Cateterismo Cardíaco/instrumentação , Pré-Escolar , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Humanos , Masculino , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Insuficiência da Valva Tricúspide/fisiopatologia
16.
Asian Cardiovasc Thorac Ann ; 27(3): 208-209, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30080103

RESUMO

Ebstein's anomaly is a rare congenital cardiac disease characterized by apical displacement of the tricuspid valve with decreased right ventricular function. Left ventricular noncompaction is a cardiomyopathy characterized by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral and apical level. We describe the very rare case of a patient living for 78 years with a benign form of Ebstein's anomaly and left ventricular noncompaction cardiomyopathy with slightly reduced systolic function.


Assuntos
Anormalidades Múltiplas , Anomalia de Ebstein , Ventrículos do Coração/anormalidades , Miocárdio Ventricular não Compactado Isolado , Valva Tricúspide/anormalidades , Idoso , Anomalia de Ebstein/diagnóstico por imagem , Anomalia de Ebstein/fisiopatologia , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Sístole , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Função Ventricular Esquerda
18.
Echocardiography ; 35(11): 1893-1894, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30295971

RESUMO

Right atrioventricular valve is historically named as tricuspid valve. However, cases depicting multi-leaflet morphology of the right atrioventricular valve were reported. Accessory leaflet of the tricuspid valve is a rare entity and associated with congenital cardiac anomalies. We present an accessory tricuspid valve in a patient with isolated persistent left superior vena cava.


Assuntos
Ecocardiografia Tridimensional/métodos , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Veia Cava Superior/anormalidades , Veia Cava Superior/diagnóstico por imagem , Adulto , Diagnóstico Diferencial , Humanos , Masculino
19.
J Pak Med Assoc ; 68(9): 1391-1393, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30317272

RESUMO

Ebstein anomaly is a rare malformation of the tricuspid valve of the heart. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. Pregnant patients with Ebstein anomaly become a challenge for the physician in terms of management. We report a case in which patient delivered successfully with Ebstein anomaly. The only complication was breathlessness. Authors concluded that women with Ebstein anomaly can have successful outcomes of pregnancy with close monitoring.


Assuntos
Anomalia de Ebstein , Administração dos Cuidados ao Paciente/métodos , Complicações Cardiovasculares na Gravidez , Valva Tricúspide , Adulto , Dispneia/diagnóstico , Dispneia/etiologia , Anomalia de Ebstein/diagnóstico , Anomalia de Ebstein/fisiopatologia , Ecocardiografia/métodos , Feminino , Monitorização Fetal/métodos , Humanos , Monitorização Fisiológica/métodos , Paquistão , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/fisiopatologia , Resultado da Gravidez , Resultado do Tratamento , Valva Tricúspide/anormalidades , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/fisiopatologia , Ultrassonografia
20.
Int J Cardiol ; 273: 230-236, 2018 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-30297190

RESUMO

BACKGROUND: To determine the impact of circulating miRNA and protein activity on the severity of ascending aortic dilatation in patients with bicuspid (BAV) and tricuspid aortic valve (TAV). METHODS: By reverse transcription polymerase chain reaction, exosomal circulating expression levels (versus healthy aorta) of miRNAs and absolute levels of transforming growth factor ß (TGF-ß), matrix metalloproteinases (MMP-2, -3 and -9), tissue inhibitors (TIMP-1, -2, -3 and -4), and soluble receptors for advanced glycation end products AGEs (sRAGE) were evaluated in ascending dilated aortas of 71 patients with different valve morphotype. RESULTS: Less-dilated ascending aorta exhibited a specific miRNA signature (i.e., miR-126 miR-15b, miR-195, miR-221, miR24, miR-30b and miR-320a), which was statistically different from that of severely-dilated ascending aorta. Among these analytes, miR-15b was the most significant (p < 0.001) and resulted as an independent predictor of aortic dilatation (ß = -1.099, p = 0.041). When patients were grouped according to aortic valve morphology, miRNAs and protein proteolytic activity were different between BAV and TAV in the expression level of miR-133a, miR-155, miR-320a, miR-34a(#000425), miR-34a(#000426), miR-494 and measurements of TGF-ß and MMP-3, MMP-9, TIMP-4. The circulating level of miR-34a(#000426) was negatively correlated to the aortic wall elasticity of bicuspid patients (R = -0.653 and p = 0.011), suggesting an apparent different mechanism of aortic wall degeneration specific for BAV. CONCLUSIONS: Taken these biomarkers together, we demonstrated that the severity of aortic size and valve morphology differently modulates miRNA analytes and protein proteolytic activity in patients with ascending aortic dilatation, and this may be useful to design new therapies that inhibit miRNAs.


Assuntos
Aneurisma Aórtico/genética , Valva Aórtica/anormalidades , Exossomos/genética , Perfilação da Expressão Gênica/métodos , Doenças das Valvas Cardíacas/genética , MicroRNAs/genética , Valva Tricúspide/anormalidades , Adulto , Idoso , Aorta/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/metabolismo , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/metabolismo , Biomarcadores/metabolismo , Estudos de Coortes , Feminino , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Valva Tricúspide/diagnóstico por imagem
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