Instituto do Coração do Hospital das Clínicas da FMUSP - São Paulo, SP
Lactente com 8 meses, do sexo masculino e de cor branca, apresentava cianose e cansaço às mamadas desde o nascimento, sem progressão. Sopro cardíaco havia sido auscultado no primeiro dia de vida. O ganho de peso foi inadequado e a dispnéia discreta em repouso se manteve inalterada. Ao exame físico estava dispnéico, com mínima cianose e os pulsos eram normais. A saturação de oxigênio era de 88%, a freqüência cardíaca 125 bpm, a freqüência respiratória 60 rpm e o peso de 6.500 g. A aorta não era palpada na fúrcula. No precórdio eram discretas as impulsões sistólicas na borda esternal esquerda e o
O eletrocardiograma mostrou sinais de sobrecarga ventricular direita com onda R de V1 de 18 mm precedida por onda Q e morfologia RS de V2 a V6. SÂP: + 30º, SÂQRS: +95º, SÂT: + 40º.
Mostra aumento da área cardíaca à custa do arco ventricular, arredondado e longo e com ponta cardíaca elevada. O arco médio é escavado, o pedículo vascular alargado por aorta dilatada e a trama vascular pulmonar aumentada, mais à direita (
Esta imagem radiográfica sugere a de cardiopatia com morfologia encontrada na tetralogia de Fallot mas em presença de hiperfluxo pulmonar, o qual por isso orienta para a atresia pulmonar com comunicação interventricular, com vasos colaterais sistêmico-pulmonares.
Esta anomalia deve ser distinguida da própria tetralogia de Fallot, esta com fluxo pulmonar aumentado em face de estenose pulmonar ainda de moderada intensidade. A atresia tricúspide e a atresia pulmonar com septo íntegro também devem ser lembradas dado o direcionamento do arco ventricular para a esquerda, simulando também aumento ventricular esquerdo.
Os elementos clínicos sugerem firmemente o diagnóstico da atresia pulmonar com comunicação interventricular em face da presença de estalido protossistólico aórtico e sopro contínuo no dorso por fluxo da esquerda para a direita através dos vasos colaterais sistêmico-pulmonares, com hiperfluxo pulmonar e cianose discreta. Ecocardiograma mostrou ao lado da atresia pulmonar e comunicação interventricular, que as artérias pulmonares tinham 3 mm de diâmetro assim como o tronco pulmonar. Cateterismo cardíaco mostrou vasos colaterais sistêmico-pulmonares dilatados partindo da aorta descendente para a árvore pulmonar direita e para o lobo inferior esquerdo. As pressões encontradas foram de AD: 8, VD: 70/8, VE: 70/8, Aorta: 70/45-53 mm Hg.
Ligadura da colateral para a direita e anastomose tipo Blalock-Taussig foram indicadas e a saturação arterial passou a 80%, em face da conseqüente diminuição do fluxo pulmonar.
Edmar Atik InCor – Av. Dr. Eneas C. Aguiar, 44 05403-000 – São Paulo, SP E-mail:
Instituto do Coração do Hospital das Clínicas da FMUSP - São Paulo, SP
Eight-month-old, white, male infant presenting with cyanosis and shortness of breath during feedings since birth, with no progression. A heart murmur had been detected on auscultation on his first day of life. Weight gain was poor and mild dyspnea at rest remained unchanged. Physical examination revealed dyspnea with mild cyanosis; pulses were normal. Oxygen saturation was 88%, heart rate was 125 bpm, respiratory rate was 60 rpm and weight was 6,500 g. The aorta was not palpable in the suprasternal notch. In the precordium, mild systolic impulses were observed on the left sternal border and the apical impulse was palpable in the fourth left intercostal space, in the midclavicular line, slightly/more forceful, limited to two fingertips. A mild early systolic click was heard in the aortic area along with a blowing, grade 1-2/6, continuous murmur in the pulmonic and aortic areas and in the posterior chest, mainly on the right side. The liver was palpable 2 cm below the right costal margin. Hb: 13.9 g/dl, Hct: 42%. The electrocardiogram showed signs of right ventricular overload with an 18-mm R wave in V1 preceded by a Q wave, and RS morphology from V2 to V6. SÂP: + 30 º, SÂQRS: +95 º, SÂT: + 40º.
The image shows enlargement of the cardiac silhouette due to a rounded long ventricular border with an upwardly displaced apex. The middle arch is excavated; the vascular pedicle is widened due to a dilated aorta, and the pulmonary vasculature is increased especially on the right side (
This image suggests a heart disease with a morphology like that found in the tetralogy of Fallot, but with the presence of increased pulmonary blood flow, which suggests pulmonary atresia with ventricular septal defect with systemic-to-pulmonary collateral vessels.
This anomaly should be distinguished from the tetralogy of Fallot itself, which presents with increased pulmonary flow because of moderate pulmonary stenosis. Tricuspid atresia and pulmonary atresia with an intact septum should also be considered given the leftward displacement of the ventricular border, also simulating left ventricular enlargement.
Clinical findings strongly suggest the diagnosis of pulmonary atresia with ventricular septal defect because of the presence of an early systolic click in the aortic area and a continuous murmur in the posterior chest due to left-to-right flow through systemic-to-pulmonary collateral vessels, with increased pulmonary blood flow and mild cyanosis. The echocardiogram showed both pulmonary arteries and pulmonary trunk with a 3-mm diameter, in addition to pulmonary atresia and ventricular septal defect. Cardiac catheterization showed dilated systemic-to-pulmonary collateral vessels from the descending aorta to the right pulmonary arterial tree and to the left lower lobe. Pressure measurements were: RA: 8, RV: 70/8, LV: 70/8, Aorta: 70/45-53 mmHg.
Ligation of the collateral to the right and Blalock-Taussig anastomosis were indicated and the arterial saturation changed to 80%, because of the resulting reduction in the pulmonary blood flow.
Edmar Atik InCor – Av. Dr. Eneas C. Aguiar, 44 05403-000 – São Paulo, SP - Brazil E-mail: