Repair of double orifice left av valve (dolavv) with endocardial cushion defect in adult

Abstract Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

Oclusão percutânea de comunicação interatrial ou de forame oval guiada por ecocardiograma intracardíaco / Percutaneous closure of atrial septal defect or foramen ovale guided by intracardiac echocardiography

O ecocardiograma transesofágico (ECO-TE) é o método mais utilizado para guiar otratamento percutâneo da comunicação interatrial (CIA) e do forame oval (FOP), mas a necessidade de um outro profissional para realizá-lo e de anestesia geral constituem inconvenientes para seu emprego. Oecocardiograma intracardíaco (ECO-IC) apresenta-se como alternativa ao ECO-TE, pois pode ser realizado pelo próprio operador e demanda apenas anestesia local, com leve ou nenhuma sedação. Nosso objetivo foi relatar a experiência do serviço com a oclusão de CIA/FOP guiada por ECO-IC. Métodos: O ECO-IC utiliza cateter de ultrassom, que é introduzido por via venosa em câmaras cardíacas direitas e, por meio de posicionamento variável do transdutor, obtém as imagens adequadas para a intervenção. Foram avaliadas as taxas de sucesso do procedimento e as complicações. Resultados: De 2011 a 2015, foram realizados 201 procedimentos guiados pelo ECO-IC, sendo 139 empacientes com CIA e 62 com FOP. A maioria dos pacientes era do sexo feminino (64,2%), as idades variaramde 7 a 78 anos (36,6 ± 19,3 anos) e o peso variou de 28 a 92 kg (62,5 ± 13,0 kg). Foram utilizadas próteses Occlutech Figulla®, e todas as intervenções tiveram sucesso, com tempos de fluoroscopia de 5,7 ± 2,4 minutos e tempo de procedimento de 21,5 ± 6,4 minutos. Dois pacientes (2,0%) apresentaram taquicardia supraventricular transitória e outros dois pacientes evoluíram com fístula arteriovenosa na via de acesso, com resolução espontânea no primeiro mês. Conclusões: O ECO-IC forneceu informações anatômicas precisas para guiar o fechamento da CIA/FOP com sucesso e eliminou as principais desvantagens do ECO-TE...

Transesophageal echocardiography (TEE) is the most widely used method to guide the percutaneous treatment of atrial septal defect (ASD) and patent foramen ovale (PFO), but the necessity of another professional to perform it and the need for general anesthesia are potential disadvantages. Intracardiac echocardiography (ICE) is seen as an alternative to TEE, as it can be performed by the interventionist and requires only local anesthesia with mild or no sedation. The aim of this study was to report our experience with ASD/PFO occlusion guided by ICE. Methods: The ICE uses an ultrasound catheter, which is intravenously inserted in the right heart chambers and acquires images for the intervention through variable positioning of the transducer. Success and complication rates of the procedure were evaluated. Results: From 2011 to 2015, 201 procedures guided by ICE were performed, comprising 139 in patients with ASD and 62 in those with PFO. Most patients were female (64.2%), ages ranged from 7 to 78 years (36.6 ±19.3 years), and weight ranged from 28 to 92 kg (62.5 ± 13.0 kg). Occlutech Figulla® prostheses were used and all interventions were successful, with fluoroscopy time of 5.7 ± 2.4 minutes and procedure time of 21.5 ± 6.4 minutes. Two patients (2.0%) had transient supraventricular tachycardia and two others had arteriovenous fistula at the access site, with spontaneous resolution in the first month of follow-up. Conclusions: ICE provided accurate anatomical information to guide the closure of the ASD/PFO and successfully eliminated the main drawbacks of TEE...

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of incomplete atrioventricular septal defect / Fatores associados à insuficiência moderada ou grave da valva atrioventricular esquerda nos primeiros 30 dias de correção de defeito de septo atrioventricular incompleto

Abstract Introduction: Left atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect. Objective: To determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect. Methods: We assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%). Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%. Results: At the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%). The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26), unlike patients without such abnormalities (P=0.016). During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02). However, after a multivariate analysis, none of the factors reached significance. Conclusion: None of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit ...

Resumo Introdução: A insuficiência da valva atrioventricular esquerda é a lesão residual mais preocupante após o tratamento cirúrgico do defeito de septo atrioventricular. Objetivo: Determinar fatores associados à insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após correção de defeito de defeito de septo atrioventricular. Métodos: Avaliamos os resultados em 51 pacientes consecutivos menores de 14 anos com defeito de septo atrioventricular incompleto, operados em nosso serviço entre 2002 e 2010. Avaliamos as seguintes variáveis: idade, peso, ausência de síndrome de Down, grau de insuficiência da valva atrioventricular esquerda antes da correção, anormalidades na valva atrioventricular e uso de anuloplastia. A mediana da idade foi de 4,1 anos e a do peso de 13,4 Kg; 37,2% tinham síndrome de Down; antes da operação, 23 apresentavam insuficiência da valva atrioventricular esquerda pelo menos moderada (45,1%); anormalidades na valva atrioventricular foram encontradas em 17,6% dos casos; anuloplastia foi realizada em 21,6% dos pacientes. Resultados: Após a correção cirúrgica, 12 casos apresentaram insuficiência da valva atrioventricular esquerda pelo menos moderada (23,5%). A variância entre os graus de insuficiência da valva atrioventricular esquerda pré e pós-operatória nos pacientes com anormalidades na valva atrioventricular não teve significância estatística (P=0,26), ao contrário daqueles sem tais anormalidades (P=0,016). Pela análise univariada, apenas a ausência de síndrome de Down teve significância estatística (P=0,02). Porém, após análise multivariada, nenhum dos fatores teve significância. Conclusão: Nenhum dos fatores estudados foi determinante de insuficiência da valva atrioventricular esquerda de grau moderado ou importante nos primeiros 30 dias após a correção de defeito de septo atrioventricular incompleto na população avaliada. Pacientes sem anormalidades na valva atrioventricular ...

Evaluación ecocardiográfica de origen anómalo de una rama arterial pulmonar desde la porción ascendente de la aorta (hemitruncus) / Echocardiographic evaluation of the anomalous origin of the pulmunary arterial branch from the ascending portion of the aorta (hemitruncus)

El origen anómalo de una de las ramas pulmonares desde la aorta ascendente en vez de ocurrir desde el tronco pulmonar, también conocido como hemitruncus constituye una singularidad cardiovascular congénita muy rara, pues ocurre en aproximadamente 0,05% de todas las forma de cardiopatías congénitas (¹). En la forma más frecuente depresentación de este defecto la rama pulmonar derecha (RPD) emerge directamente de la aorta ascendente en un 70%-80% de los casos (²). La ecocardiografía con Doppler color es el método de mayor utilidad para realizar el diagnóstico, los cortes apicales, paresternales y subcostales en eje corto de grandes vasos así como las visiones supraesternales longitudinales son esenciales para conseguir una completa evaluación del defecto. Presentamos una secuencia de imágenes ecocardiográficas de una paciente que es referida tardíamente a nuestro centro para evaluación cardiológica con historia de cianosis desde el nacimiento, fatiga y episodios repetidos de hemoptisis que resultó ser portadora de un origen anómalo de rama derecha de arterial pulmonar emergiendo desde la aorta ascendente y que desarrolló hipertensión arterial pulmonar en rangos suprasistémicos con resistencia vascular pulmonar también muy elevada.

Anomalous origin of one pulmonary branch from the ascending aorta rather than occurring from the pulmonary trunk, also known as hemitruncus is a very rare congenital cardiovascular entity, which occurs in approximately 0.05% of all forms of congenital heart disease (¹). In the most common presentation of this defect, the right pulmonary branch (RPB) emerges directly from the ascending aorta in 70%-80% of cases (²). Color Doppler echocardiography is the most useful method to establish the diagnosis. Apical, subcostal, and parasternal short axis views of the great vessels and longitudinal suprasternal views are essential for a complete assessment of the defect. We present a sequence of echocardiographic images of a patient referred late to our center for a cardiac evaluation with a history of cyanosis from birth, fatigue and repeated episodes of hemoptysis that resulted from having an anomalous origin of the right pulmonary artery branch emerging from the ascending aorta with development of pulmonary arterial hypertension exceeding systemic levels along with very high pulmonary vascular resistance.

Evolución clínica en pacientes con síndrome de Down y cardiopatía congénita / Clinical outcome in Down syndrome patients with congenital heart disease

Introducción: La evolución a largo plazo de pacientes con síndrome de Down y cardiopatía congénita es poco conocida. Material y métodos: Estudio retrospectivo de pacientes con síndrome de Down y cardiopatía congénita y pacientes con defecto del tabique auriculoventricular y sin trisomía 21. Resultados: Entre 2004 y 2008 se hizo seguimiento a 317 pacientes con cardiopatía congénita; 19 pacientes (6%) con edad media de 26.8 ± 8.1 años tenían síndrome de Down. Las cardiopatías congénitas más frecuentes fueron el defecto del tabique auriculoventricular (63%) y la comunicación interventricular (26%); 10 (53%) habían sido intervenidos quirúrgicamente en la infancia, tres de ellos con defecto auriculoventricular completo requirieron reintervención en la edad adulta (dos por obstrucción al tracto de salida del ventrículo izquierdo y uno por insuficiencia severa de la válvula auriculoventricular izquierda); cuatro (21%) desarrollaron hipertensión arterial pulmonar, con mejoría del grado funcional al recibir tratamiento con bosentan; dos (10.5%) tuvieron endocarditis bacteriana; dos (10.5%) fallecieron. No existieron diferencias en el grado de insuficiencia de la válvula auriculoventricular izquierda (1.5 ± 0.9 versus 1.7 ± 0.8, p = 0.689) entre pacientes con defecto auriculoventricular con y sin síndrome de Down. Conclusiones: La insuficiencia de la válvula auriculoventricular izquierda y la obstrucción al tracto de salida del ventrículo izquierdo fueron las causas más frecuentes de reintervención en la edad adulta en pacientes con defectos del tabique auriculoventricular.

BACKGROUND: Long-term complications of Down syndrome patients with congenital heart disease are poorly known. METHODS: We carried out a retrospective study of Down syndrome patients with congenital heart disease and patients with atrioventricular septal defect with and without Down syndrome. RESULTS: Between 2004 and 2008, 317 patients with congenital heart disease were followed-up in the Adult Congenital Heart Disease Unit. Of these patients, 19 (6%) with a mean age of 26.8 +/- 8.1 years had Down syndrome. Atrioventricular septal defect was the most frequent congenital heart disease(63%) followed by ventricular septal defect (26%). Ten patients (53%) were operated on during childhood. Three of these patients required reoperation during adulthood (two patients due to left ventricle outflow tract obstruction and one patient due to left atrioventricular valve insufficiency). Four patients (21%) had Eisenmenger syndrome with improvement of functional class in those treated with bosentan, two patients (10.5%) had bacterial endocarditis and two patients (10.5%) died. No significant differences were seen in left atrioventricular valve insufficiency between atrioventricular septal defect in patients with and without Down syndrome (1.5 +/- 0.9 vs. 1.7 +/- 0.8, p = 0.689). CONCLUSIONS: Left atrioventricular valve insufficiency and left ventricle outflow tract obstruction were the most frequent long-term complications requiring surgical reintervention in patients with atrioventricular septal defect.

Defeito completo do septo atrioventricular com cianose / Complete repair in total atrioventricular canal defect with cyanosis / Complete repair in total atrioventricular canal defect with cyanosis

Os defeitos do septo atrioventricular total (DSAVT) representam 4 por cento das mal formações cardíacas e acima de 50 por cento dos defeitos observados na síndrome de Down (SD)¹. A apresentação clínica é de insuficiência cardíaca precoce na infância e hipertensão pulmonar por hiperfluxo. Raramente a cianose é observada e sugere hipertensão pulmonar ou associação à tetralogia de Fallot³, dupla via de saída de ventrículo direito², anomalia de Ebstein4, drenagem anômala de cava esquerda persistente em átrio esquerdo (Barbero Marcial, comunicação pessoal). Crianças com SD são particularmente difíceis de avaliação por apresentarem obstrução de vias aéreas superiores, que podem contribuir com o aumento da resistência pulmonar observada no cateterismo cardíaco. A presença de cianose pré-operatória constitui-se um desafio ao tratamento cirúrgico devido ao risco de hipertensão pulmonar irreversível com falência ventricular direita com, a correção dos defeitos intracardíacos.

Atrioventricular septal defects account for 4 percent of congenital cardiac malformations and over 50 percent of cardiac defects seen in Down syndrome¹. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle², Ebstein anomaly³, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction4, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.

Multiple systemic emboli complicating the course of a patient with an atrial septal defect, an atrial septal aneurysm and an endocardial right atrial pacemaker lead

We describe an adult patient with a large atrial septal defect, an atrial septal aneurysm and thrombus formation on a transvenous right atrial pacing lead. Because of right-to-left shunting through the atrial septal defect, she developed multiple systemic emboli to the spleen and left kidney (with infarcts), to the left leg, and probably to the brain as a cerebrovascular accident. The fundamental guiding principle of avoiding endocardial pacing leads in patients with congenital intracardiac communications, was violated in this patient, leading to serious dire consequences and complications.

Defeito total do septo atrioventricular: correlaçäo anatomofuncional entre pacientes com e sem Síndrome de Down / Complete atrioventricular canal: clinical anatomic correlations between patients with and without Down's Syndrome

OBJETIVOS: Comprarar o grau de repercussäo, fatores responsáveis e época de aparecimento dos sintomas do defeito do septo atrioventricular (DSAV), em pacientes com e sem síndrome de Down...

Defeito parcial do septo atrioventricular associado a doença de Ebstein da valva atrioventricular direita / Partial Atrioventricular Septal Defect Associated with Right Atrioventricular Valve Ebstein's Disease

A associaçäo de doença de Ebstein e defeito septal atrioventricular é extremamente rara, havendo 13 casos na literatura e nenhum em nosso meio. Descrevemos uma paciente de 15 anos com diagnóstico eco e angiográfico e confirmaçäo cirúrgica. A näo identificaçäo de uma das lesöes poderia comprometer o planejamento e resultado cirúrgico

Early experience with the two patch technique for complete atrioventricular septal defect.

A total of 12 cases underwent repair of complete atrioventricular (AV) septal defect utilising the two-patch technique. There were 4 males and 8 females. The mean age at repair was 9.6 months (range, 3 to 49 months). The average weight was 5.4 kg (range, 3.5 to 13 kg). Five had associated patent ductus arteriosus. A Gore-Tex patch and glutaraldehyde preserved pericardium was utilised for the ventricular and atrial portions of the defects respectively in all patients. Four of these were done under hypothermic circulatory arrest. There were no intra-operative deaths. Early mortality comprised of 2 patients (2/12, 16.6%). One due to a pulmonary hypertensive crisis and the other to septicemia. The mean duration of ventilatory support was 62 hours (range, 24 to 192 hours). The mean duration of inotropic support was 57 hours (range, 24 to 192 hours). Primary repair of AV septal defects using the two-path of technique can be accomplished with a low early mortality in carefully selected patients.

Estenose pulmonar congênita e tuberculose: apresentaçäo de 3 casos / Congenital pulmonary stenosis and tuberculosis: report of 3 cases

Säo apresentados 3 casos com estenose pulmonar congênita, defeitos septais intracardíacos e tuberculose. Em um caso, com estenose pulmonar valvar e defeito do septo atrial, "shunt" direito-esquerdo e severa cianose, a tuberculose manifestou-se muito discreta, afetando ambas as pleuras e o ápice do pulmäo direito, antes da valvotomia e fechamento da comunicaçäo interatrial. O exame radiológico praticado 1 ano e 5 meses após demonstrou lobite superior direita com escavaçöes e a baciloscopia foi positiva. Em outro caso, praticou-se anastomose Blalock-Taussig numa criança de 5 anos com estenose pulmonar infundibular associada à comunicaçäo interventricular e a infecçäo tuberculosa manifestou-se no exame radiológico praticado quando o paciente atingiu 18 anos de idade, observando-se na radiografia lesäo cavitária no ápice esquerdo. No terceiro caso, com estenose pulmonar valvar e defeito do septo ventricular, "shunt" bidirecional entre os ventrículos e ausência de cianose, a tuberculose instalou-se como processo primária agudo (quadro pneumônico) no final do primeiro mês de vida. Em seguida, tornou-se crônico e causou fibrotórax esquerdo, com forte retraçäo costofrênica mediastinal, conforme radiografias obtidas aos 7 anos de idade. Consideraçöes quanto a freqüência, hipótese sobre a predisposiçäo para a tuberculose nos caso com estenose pulmonar congênita, isolada ou associada com outras malformaçöes, sua importância na indicaçäo da cirurgia e na evoluçäo pós-operatória, säo feitas de acordo com a literatura ao alcance