Evolutional aspects of children and adolescents with surgically corrected aortic coarctation: clinical, echocardiographic, and magnetic resonance image analysis of 113 patients.

OBJECTIVE: The goals of this study were to determine the prevalence of hypertension and recoarctation in operated children and teenagers and to integrate clinical and imaging technique data. METHODS: One hundred thirteen infants and children (ages 14 years or less) were retrospectively divided into 3 groups according to the age at operation and the surgical technique: 79 underwent resection with end-to-end anastomosis; 14 had patch enlargement; 13 had subclavian flap aortoplasty; and 7 had other techniques performed. The mean age at operation was 3.95 +/- 4.17 years and the mean follow-up period was 4.62 +/- 4.90 years. Each patient was clinically examined and Doppler echocardiography was performed in 112 patients. Sixty-six patients underwent magnetic resonance imaging. Diameters of aortic arch were measured at 4 levels; the ratios between each one and descendent aorta diameters were calculated. Qualitative variables and associations were studied by Fisher exact test or chi-squared test. Comparisons of measurements in different groups were performed using variance analysis, with tests of selective contrasts (nonparametric tests). The level of statistical significance was <.05. RESULTS: The prevalence of hypertension and recoarctation was 38% and 14%, respectively. No statistical difference was found among the age groups. In 65 patients who underwent magnetic resonance imaging, the transverse aortic arch was hypoplastic in 31 (47%) patients; 41 (63%) had stenosis at the site of anastomosis (ratio < 0.9). CONCLUSION: Hypoplasia of transverse aortic arch was highly prevalent. These data suggest that hypoplastic aortic arch should be corrected concomitantly with coarctation.

Endomyocardial fibrosis in infancy.

The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age.

Endomiocardiofibrose em lactente / Endomyocardial fibrosis in infancy

The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age

Arterial hypertension in a child with Williams-Beuren syndrome (7q11.23 chromosomal deletion).

We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment.

Hipertensäo arterial em criança portadora de síndrome de Williams-Beuren (deleçao cromossômica 7q11.23) / Arterial hypertension in a child with Williams-Beuren syndrome (7q11.23 chromosomal deletion)

We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed. Systemic vascular alterations caused by deletion of the elastin gene may occur early in individuals with Williams-Beuren syndrome, leading to the clinical manifestation of systemic arterial hypertension refractory to drug treatment

Anomalia de UhL. Diagnóstico diferencial e indicaçäo de transplante cardíaco em lactente / Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed

Transplante cardíaco no neonato e na infância: resultados a médio prazo / Heart transplantation in neonates and children: intermediate-term results

OBJECTIVE: To assess intermediate-term outcome in children who have undergone orthotopic heart transplantation. METHODS: We carried out a longitudinal and prospective study between October '92 and June '99 comprising 20 patients with ages ranging from 12 days to 7 years (mean of 2.8 years). We employed a double immunosuppression protocol with cyclosporine and azathioprine and induction therapy with polyclonal antithymocyte serum. Survival and complications resulting from the immunosuppression protocol were analyzed. RESULTS: The double immunosuppression protocol and the induction therapy with polyclonal antithymocyte serum resulted in an actuarial survival curve of 90 per cent and 78.2 per cent at 1 and 6 years, respectively, with a mean follow-up period of 3.6 years. One patient died due to acute rejection 40 days after transplantation; another patient died 2 years after transplantation due to lymphoproliferative disorder; a third patient died because of primary failure of the graft; and a fourth patient died due to bronchopneumonia. The major complications were as follows: acute rejection, infection, nephrotoxicity, and systemic hypertension. The means of rejection and infection episodes per patient were 2.9 and 3.4, respectively. After one year of transplantation, a slight reduction in the creatinine clearance and systemic hypertension were observed in 7 (38.9 per cent) patients. CONCLUSION: Heart transplantation made life possible for those patients with complex congenital heart diseases and cardiomyopathies in refractory congestive heart failure constituting a therapeutical option for this group of patients in the terminal phase.

Estenose aórtica valvar crítica no recém-nascido. Avaliaçäo clínica e resultados cirúrgicos em 18 pacientes / Critical aortic stenosis in the neonate. Clinical assessment and surgical outcome in 18 patients

OBJECTIVE - To assess neonates with aortic stenosis with early decompensation operated upon. (LCO) (CHF). METHODS - A and retrospective study analyzing 6 neonates with LCO, group I (GI), and 12 neonates with CHF, group II (GII). Clinical radiographic, electrocardiographic and echocardiographic findings also provided comparative bases for the study, as did surgical and evolutional findings. RESULTS - The mean ages at hospitalization and surgery (p = 0.0031) were 14.3 and 14.8 days in GI and 35.4 and 42.8 days in GII, respectively. Cardiac murmurs were more intense in GII (p = 0.0220). The aortic ring was smaller in GI (8.0 2.5mm) as compared to GII (11.4_1.4mm) (p = 0.2882). Vntricular function was reduced to 18_5.5 percent and 3.3_7.6 percent in GI and GII, respectively (p = 0.0162). Artic atresia, however, was present only in 2 neonates in GI. Five of 6 patients in GI died but all patients in GII survived (p=0.0007). In the latter group, 84.6 percent of the patients were infunctional class I (FC-I) in the long-term follow-up, with moderate residual lesions in 6 neonates, discrete residual lesions in 4, and reoperation in 2. CONCLUSION - Aortic stenosis is a severe anomaly of the neonate, whose immediate evolution depends on the pre-operative anatomic and functional findings, and the late evolution essentially depends on the anatomic features of thevalve

Janela aortopulmonar: análise clínico-cirúrgica de 18 casos / Aortopulmonary window: clinical and surgical assessment of 18 cases

O objetivo aortopulmonar (JaoP) é uma anomalia congênita incomum cujos achados clínicos dependem do tamanho do defeito e das lesões associadas. Assim, avaliamos a nossa experiência, comparando-a com 296 casos relatados na literatura. Métodos - Estudo retrospectivo de 18 pacientes (idade variando de 13 dias a 31 anos, sendo 13 (72,2 por cento) do sexo feminino), divididos em dois grupos: grupo A (GA): 10 pacientes com defeito isolado e grupo B (GB): 8 pacientes com defeitos associados. Resultados - Insuficiência cardíaca ocorreu em 14 pacientes, principalmente no GA (8 pacientes), e cianose em três, dois no GB (tetralogia de Fallot (TF) e dupla via de saída de ventrículo direito (DVSVD)) e um com hipertensão pulmonar. Em 5 (38,4 por cento) pacientes do GA, foi diagnosticado insuficiência mitral devido ao sopro sistólico e à sobrecarga do VE ao ECG. No GB, os achados clínicos dependiam dos defeitos associados. O diagnóstico pela ecocardiografia foi feito em 11 (61,2 por cento) pacientes, sendo confundido com insuficiência mitral em três, com canal arterial em um e mascarado por defeitos importantes do tipo DVSVD (2 casos) e TF (1 caso). O diagnóstico foi realizado pelo cateterismo em 3 (16,6 por cento) pacientes, pela cirurgia em 3 (16,6 por cento) e pela necropsia em um (5,5 por cento). Foi feita sirurgia em 14 (77,7 por cento) pacientes com um óbito imediato, e boa evolução a longo prazo nos demais. Conclusão - O diagnóstico de JaoP pode ser confundido com outros defeitos. Os achados clínicos aliados ao estudo e ecocardiográfico podem orientar o diagnóstico do defeito, se analisados adequadamente.

Ausência de conexäo atrioventricular à direita. Apresentaçäo morfológica e clínica quando o ventrículo principal é morfologicamente direito / Absence of the right atrioventricular connection. Morphological and clinical presentation when the dominant ventricle is morphologically right

Ausência de conexão atrioventricular à direita com o ventrículo dominante, morfologicamente direito, colocado à esquerda, em conexão com o átrio esquerdo, é uma rara situação. Os autores apresentam cinco casos com esta disposição morfológica, salientando os aspectos anatômicos e clínicos, bem como a importância da nomenclatura baseada na análise segmentar seqüencial.

Avaliaçäo hemodinâmica e evoluçäo clínica de crianças portadoras de cardiomiopatia dilatada grave candidatas a transplante cardíaco / Hemodynamic evaluation and clinical outcome of children candidates for heart transplantation with severe dilated cardiomyipathy

Objetivo - Estudar o perfil dos parâmetros hemodinâmicos e a evolução clínica de crianças candidatas a transplante cardíaco, portadoras de cardiomiopatia grave. Métodos - Foram 24 crianças, com idade entre 4 meses e 10 anos e 8 meses (média de 3,7+2,5 anos), no período de fevereiro/92 a maio/96, submetidas a estudo hemodinâmico e medidos os seguintes parâmetros: débito cardíaco, pressão média de artéria pulmonar (PMAP) e pressão capilar pulmonar. Foram calculados o índice de resistência vascular pulmonar (IRVP) e gradiente de pressão transpulmonar (GPT). Resultados - Do ponto de vista evolutivo, 10 (41,6 por cento) crianças foram transplantadas (grupo A), 5 (20,8 por cento) aguardam o transplante (grupo B) e 9 (37,6 por cento) faleceram (grupo C). Observou-se que a média das idades dos pacientes do grupo B foi significativamente menor que do grupo C. Dos dados hemodinâmicos, a PMAP, GTP e IRVP apresentaram médias significativamente menores no grupo A em relação ao grupo C. Conclusão - O perfil hemodinâmico de crianças candidatas ao transplante cardíaco mostrou-se compatível ao quadro clínico de insuficiência cardíaca grave. A idade foi o único fator que diferenciou o grupo B e C (p=0,036). O IRVP, PMAP e o GTP foram fatores que diferenciam de modo significativo o grupo A e o grupo C (p=0,010; p=0,044 e p=0,023, respectivamente). Quanto maior a idade no momento da indicação do transplante na criança, pior foi seu prognóstico.

Origem anômala pulmonar da artéria coronária esquerda com trajeto intramural aórtico: diagnóstico e tratamento cirúrgico / Pulmonary anomalous origin of the left coronary artery with an intramural aortic route: diagnosis and surgical treatment

Cinco pacientes com idades entre 3 meses e 11 meses, com diagnóstico clínico, ecocardiográfico e angiográfico de origem anômala da artéria coronária esquerda do tronco pulmonar foram operados no período de 1987 a 1997. Somente durante o ato operatório foi diagnosticado o trajeto intramural aórtico. Em 4 deles uma nova técnica cirúrgica foi utilizada e em 1 deles o reimplante da coronária esquerda foi empregado. Nesta técnica, após o diagnóstico intra-operatório do trajeto intramural, o tronco pulmonar é aberto e a coronária esquerda é explorada com um probe de modo a identificar seu trajeto. A seguir, a aorta é aberta e o segmento intramural da ocorrência é aberto longitudinalmente. A camada íntima da coronária é suturada à íntima e média da aorta, criando-se uma ampla janela aortocoronária. Por último, a origem da coronária anômala no tronco pulmonar é ligada. Os 4 pacientes operados com esta técnica evoluíram com melhora da classe funcional e da funçao contrátil ao ecocardiograma. Apresentam-se em classe funcional grau I (NYHA) após um período de seguimento de 7 meses a 10 anos. O único óbito ocorreu no paciente submetido a dissecçao da coronária esquerda e reimplante na aorta, que apresentou complicaçoes pós-operatórias, falecendo 6 meses após a alta hospitalar. Nestes casos, a alternativa de espera a coronária da parede aórtica e reimplantar a coronária deve ser evitada. A técnica descrita permite a correçao cirúrgica desta anomalia de maneira simples e segura, com bons resultados a longo prazo.

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...

Dupla via de saída do ventrículo direito com comunicaçäo interventricular näo relacionada: resultados da correçäo cirúrgica com técnica de múltiplos retalhos / Double-outlet right ventricle with non-committed ventricular septal defect: surgical results using the multiple patches technique

Objetivo: Apresentar uma modificaçäo técnica para a correçäo cirúrgica biventricular da dupla via de saída de ventrículo direito com comunicaçäo interventricular näo relacionada. Métodos: No período de abril de 1987 a fevereiro de 1996, 15 pacientes portadores desta anomalia foram submetidos à técnica de reconstruçäo biventricular com "tunelizaçäo do ventrículo esquerdo para a aorta utilizando-se 2 ou mais retalhos de pericárdio bovino. A idade variou entre 2 a 13 anos (média = 4,8 anos; média = 5 anos). Treze pacientes apresentavam situs solitus e levocardia, 1 paciente situs inversus e dextrocardia e 1 paciente situs solitus e dextrocardia. A construçäo do túnel inicia-se no átrio direito. Sendo a CIV pequena ou restritiva, é feita a ampliaçäo em sentido anterior e o primeiro retalho é suturado na borda póstero-inferior do defeito direcionando-o para o septo, em posiçäo anterior e superior. A seguir, através de ventriculotomia direita o segundo, terceiro e, por vezes, o quarto retalhos säo suturados direcionando o túnel para o anel aórtico. Resultados: Houve 3 óbitos (mortalidade de 20 por cento), 2 hospitalares, 1 tardio e os 12 pacientes restantes foram seguidos por um período variável de 10 meses a 9 anos (média 33 meses) encontrando-se todos em classe funcional I (NYHA). Comunicaçäo interventricular residual, mínima, foi observada em 1 paciente, estenose pulmonar residual em 2 pacientes e insuficiência pulmonar em 1 paciente. Em nenhum dos pacientes demonstrou-se obstruçäo do túnel ventrículo esquerdo - aorta. Conclusäo: Baseados nestes resultados, concluímos que esta técnica para reparo biventricular permite a construçäo de um túnel com diâmetro interno adequado, respeitando as mudanças de direçäo existentes entre a comunicaçäo interventricular e a aorta. O túnel de pericárdio bovino ocupa menor espaço intraventricular causando, portanto, menor risco de obstruçäo da via de saída de ventrículo direito.

Sling da artéria pulmonar / Pulmonary artery sling

The authors present a case of a two-year-old boy with stridor since birth, and also recurrent episodes of pulmonary infections, due to mechanical compression of distal trachea by a left pulmonary artery sling. They discussed diagnostic approach, surgical management and review of the literature. To our knowledge, it is the first case of sling of the pulmonary artery reported in Brazil and the fifth in a latin language country.

Quantificação sistemática das artérias em biópsias pulmonares de pacientes com defeitos cardíacos congênitos e sua contribuição para a indicação e abordagem terapêutica / Systematic Quantification of the Arteries in Lung Biopsies of Patients with Congenital Heart Defects and its Contribution to the Therapeutic Indication / Systematic quantification of the arteries in lung biopsies of patients with congenital heart defects and its contribution to the therapeutic management

PURPOSE: To determine the value of the quantitative analysis of lung biopsies from patients with congenital cardiac defects. METHODS: Fourty nine biopsies were examined, from patients: 43 patients increased pulmonary blood flow, 3 with pulmonary atresia and large systemic-to-pulmonary collateral vessels, and 3 with decreased pulmonary flow. The degree of lesion was determined as in Heath-Edwards and of Rabinovitch and col. RESULTS: The Heath and Edwards grade was determined in 41 cases; I in nine; II in 23; III in eight; IV in one; 3 biopsies showed evidence of reduced pulmonary flow and 5 had no signs of vascular disease. The Down patients (7) presented a greater proportion of severe lesions. Quantitative evaluation was obtained in 35 biopsies: 11 had grade B and 24 had grade C. Wall atrophy and dilatation of intraacinar arteries were detected in 7 cases, what suggested the existence of obstructive lesions in proximal vessels, even if not sampled. Medical thickness greater than 2 times the normal were observed in pre-acinar arteries from 14 biopsies. CONCLUSION: The morphometric approach allowed us to detect severe lesions which the qualitative analysis alone would not indicate adequately. In the patients presenting decreased pulmonary flow, morphometry made possible to assess if the degree of arterial wall hypertrophy was compatible with a surgery of atrio-pulmonary anastomosis.