Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center

Abstract Objective: To evaluate surgical management and results of patients with pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries (PA/VSD/MAPCAs). Methods: We reviewed a consecutive series of patients with PA/VSD/MAPCAs between January 2012 and October 2018. Study patients were separated into Group A, efficient MAPCAs; Group B, hypoplastic MAPCAs; Group C, severe hypoplastic MAPCAs at all divisions; and Group D, distal stenosis at most MAPCAs divisions. Results: Thirty-six patients were included in the study. Median age at operation time was 5.5 months (2-110 months), median weight was 8 kg (2.5-21 kg), and median number of MAPCAs was three (1-6). In Group A, 14 patients underwent single-stage total correction (TC); in Group B, 18 patients underwent unifocalization and central shunting; and in Group C, four patients had aortopulmonary window creation and collateral ligation. No patient was placed in Group D. Seventy percent of patients (n=25) had the TC operation. Early mortality was not seen in Group A, but the other two groups had a 13.6% mortality rate. At the follow-up, three patients had reintervention, two had new conduit replacement, and one had right ventricular outflow tract reconstruction. Conclusion: Evaluating patients with PA/VSD/MAPCAs in detail and subdividing them is quite useful in determining the appropriate surgical approach. With this strategy, TC can be achieved in most patients. Single-stage TC is better than other surgical methods due to its lower mortality and reintervention rates. Care should be taken in terms of early postoperative intensive care complications and reintervention indications during follow-ups.

Clinical and Epidemiological Profiles of Patients Admitted to a Pediatric Cardiac Intensive Care Unit

Abstract Background: Congenital and acquired heart diseases are important causes of morbidity and mortality in children. In critical congenital heart defects, when treatment is not adequate, clinical manifestations may lead to death in the neonatal period. Objective: To establish the clinical and epidemiological profile of patients admitted to the pediatric cardiac intensive care unit (UTI) in a tertiary hospital. Methods: This was a cross-sectional study conducted from January 2013 to December 2014, based on analysis of patients' medical records. The study sample was composed of 307 children and adolescents with congenial and acquired heart diseases. The score Risk Adjustement for Congenital Heart Surgery 1 (RACHS-1) was used for categorization of the various surgical procedures. Descriptive statistics were calculated using the Satistical Package for Social Sciences (SPSS). Categorical variables were compared using the Pearson's chi-square test, considering a level of significance of 5%. Results: There was a predominance of patients aged between 28 days and one year (44%). Congenital heart diseases (91.9%) prevailed over acquired heart diseases (8.1%). Extracorporeal circulation was used in 138 patients who underwent surgical procedures, lasting from 12 to 261 minutes. Most patients (88.9%) were discharged from the ICU and 11.1% died. Using the score RACHS-1, corrective cardiac surgery was performed in 75.8% and paliative surgery in 24.2% of the patients. Conclusions: Patients aged between 28 days to one year, with cyanotic congenital heart disease, undergoing cardiac surgery with extracorporeal circulation duration longer than 120 minutes are at a higher risk of death.

Relato de caso sobre comunicação interventricular pós-infarto agudo do miocárdio / Case report on acute postinfarction myocardial interventricular septal rupture

O presente artigo relata dois casos de pacientes, os quais foram admitidos na emergência do Instituto de Moléstias Cardiovasculares (IMC) com dor tipicamente anginosa, caracterizada como dor retroesternal em aperto, com irradiação para a face medial do braço esquerdo e relacionada ao esforço, com melhora no repouso. No exame físico, apresentavam taquicardia e sudorese. O eletrocardiograma evidenciou sinais de isquemia miocárdica em ambos os pacientes. Após as medidas iniciais serem tomadas, um dos pacientes foi submetido à terapia trombolítica e o outro à angioplastia para implantação de stent . Mais tarde, foi identificada a comunicação interventricular (CIV), seguido da necessidade de correção cirúrgica da mesma. Ambos os pacientes evoluíram bem, apesar da alta taxa de mortalidade desta condição

This paper reports two cases of patients who were admitted to the emergency room of the Instituto de Moléstias Cardiovasculares (IMC) with typically anginal pain, characterized by tight retrosternal pain radiating to the inside left arm, related to stress and improving with rest. Physical examination showed tachycardia and excessive sweating. Electrocardiogram showed signs of myocardial ischemia in both patients. After the initial measures were taken, one of the patients received thrombolytic therapy, and the other angioplasty for stent implantation. Later, interventricular septum rupture (IVSR) was identified, followed by the need for surgery to correct it. Both patients recovered well, despite the high mortality rate of this condition

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.

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 ...

Fechamento do Canal Arterial Persistente Tipo Janela Com o Oclusor Septal AMPLATZER® / Percutaneous Closure of Window-Type Patent Ductus Arteriosus With the AMPLATZERTM Septal Occluder

Há vários anos, a oclusão percutânea do canal arterial persistente é uma técnica factível e eficaz na maioria das variantes morfológicas descritas por Krishenko. O tipo B, em janela, caracterizado por ser curto, permanece um desafio, devido ao maior risco de embolizações das próteses e das oclusões incompletas. Descrevemos aqui o uso bem-sucedido de oclusores septais AMPLATZER® em três pacientes com canal arterial em janela, dois casos tratados com dispositivos de 5 mm e um com o de 7 mm. O dispositivo AMPLATZER® desenhado para a oclusão da comunicação interatrial mostrou-se eficaz para o tratamento percutâneo dessa variante morfológica de canal arterial persistente.

For several years the percutaneous closure of patent ductus arteriosus has been a reliable and effective technique for most of the morphologic variants described by Krichenko. Type B, or window-type, patent ductus arteriosus remains a challenge due to the higher risk of device embolizations and incomplete occlusions. We report the successful use of AMPLATZERTM septal occluder in three patients with window-type patent ductus arteriosus, two cases treated with a 5-mm device and one case with a 7-mm device. The AMPLATZERTM device designed for the occlusion of atrial septal defects is effective for the percutaneous treatment of this morphological variant of patent ductus arteriosus.

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.

Effects of modified ultrafiltration on blood products requirement in children undergoing open heart surgery

To find out the effects of modified ultrafiltration on blood products requirement for transfusion in congenital heart disease children after open heart surgery.This was a quasi-experimental study between two clinical groups. Patients were assigned to both groups by using convenient sampling; to do Modified Ultrafiltration or not was surgeon's preference who was unaware whether the patient is participating in any study or not. The study was carried out at Armed Forces Institute of Cardiology/National Institute of Heart Diseases [AFIC/NIHD] Rawalpindi between August, 2005 and September, 2006. Total 200 patients were included in this study and were divided equally into two groups; study group [MUF] and control group [non MUF] keeping hundred patients in each group. Significantly increased level of hemoglobin after MUF [9.7 +/- 1.4 gm/dl before MUF versus 13.6 +/- 1.6 gm/dl after MUF, p<0.001] and significantly decreased volume of blood products required for transfusion in study group [24.1 +/- 24.5 ml/kg versus control: 43.81 +/- 42.4 ml/kg, p<0.001]. Significantly increased hemoglobin level was observed during first three days of ICU stay [12.6 +/- 1.8 g/dl versus control: 11.6 +/- 2.1 g/dl, p=0.001on first postoperative day, 11.3 +/- 1.8 g/dl versus control: 10.8 +/- 1.9 g/dl, p=0.039 on second postoperative day and 11.3 +/- 1.5 g/dl versus control: 10.5 +/- 1.8 g/dl, p=0.022 on third postoperative day]. From this study we concluded that use of MUF is well tolerated in all the patients and due to removal of extra water from patients circulation after separation from CPB resulted in hemodynamic benefits, significantly less use of blood products and better postoperative hemoglobin and hematocrit management

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

Tratamento cirúrgico da ectopia cordis: relato de três casos e revisäo da literatura / Surgical treatment of ectopia cordis: report of 3 cases and review of the literature

Entre 1985 e 1990, três neonatos portadores de ectopia cordis (EC) foram admitidos no InCor - FMUSP; dois com defeito do tipo tóraco-abdominal e um do tipo torácico. Todas as crianças tinham anomalias cardíacas associadas: atresia tricúspide tipo I-A, ausência de artérias pulmonares centrais e colaterais sistêmico-pulmonares (um paciente); comunicaçao interventricular (CIV) e comunicaçao interatrial (CIA) (um paciente); CIA (um paciente). Uma criança nao foi operada, devido a infecçao e laceraçao da artéria pulmonar, indo a óbito por sangramento. As duas outras foram operadas, na tentativa de se recobrir o coraçao com pele; uma foi a óbito no 1( dia de pós-operatório, por baixo débito (BD); a outra teve evoluçao mais longa, indo a óbito no 141( dia de pós-operatório, causado por insuficiência respiratória. A longa evoluçao desse último paciente deveu-se a um defeito cardíaco de pouca repercussao hemodinâmica (CIA) e à colaboraçao de um cirurgiao plástico na equipe cirúrgica. Este trabalho relata esses três casos e faz uma suscinta revisao da literatura.

Rinosseptoplastia funcional precoce / Early functional rhinoseptoplasty

Os autores estudam genericamente a fisiologia nasal, dando ênfase à patologia do septo e cornetos. Sob certos aspectos, consideram a importância dos cornetos maior que a do septo, no que se refere à fisiologia da respiraçäo. Acham que se pode operar crianças a partir dos 12 anos, desde que se respeite a integridade da "moldura" da cartilagem do septo que, sendo hialina, tem o seu crescimento assim garantido. Acham que o pericôndrio näo é täo importante para o crescimento da cartilagem do septo e cartilagens hialinas em geral, como é o periósteo para os ossos

Correçäo das deformidades estéticas nasais e dos desvios septais em tempo único / Treatment of esthetic deformities of the nose and septal deformities in a single session operation

É apresentado uma análise de 78 pacientes submetidos a rinoplastia, no período de março de 1985 a dezembro de 1989, destacando-se a importância do tratamento dos desvios septais conjuntamente com a rinoplastia estética. Dos 78 pacientes, 31 apresentavam diversos graus de obstruçäo nasal, sendo 26 submetidos a tratamento cirúrgico, empregando diferentes procedimentos, dependendo de cada caso em particular. Salienta-se a importância de um estudo pré-operatório detalhado que inclua avaliaçäo radiológica, estudo constrastado do perfil facial, rinoscopia, documentaçäo fotográfica e, nos pacientes que apresentam alguma alteraçäo da dinâmica do fluxo nasal, um estudo rinomanométrico. Nestes 31 pacientes, constataram-se graus variáveis de obstruçäo nasal devido a: desvios septais, desvios dos ossos nasais e/ou hipertrofia dos cornetos associada ou näo a alteraçöes alérgicas. A avaliaçäo precisa ofereceu dados que definiram as alteraçöes anatômicas para os diversos tipos de obstruçäo, possibilitando escolher a melhor opçäo cirúrgica para cada caso. Finalmente é enfatizado a importância do conhecimento anatômico e funcional do nariz para a realizaçäo de cirurgias nasais, a utilizaçäo de material adequado para os procedimentos cirúrgicos, sendo que uma única cirurgia possibilita a correçäo das deformidades e das obstruçöes nasais, e que a avaliaçäo pós-operatória da obstruçäo é grande parte subjetiva