Relatamos o caso de um adolescente encaminhado com o diagnóstico de hipertensão pulmonar. A investigação não invasiva detectou comunicação interatrial seio venoso com sinais de hipertensão pulmonar. No estudo hemodinâmico o diagnóstico foi confirmado, sendo também notada compressão esquerda pelo tronco pulmonar. O paciente foi submetido à oclusão cirúrgica da comunicação interatrial e à plastia redutora do tronco pulmonar. Dois anos após o procedimento, o paciente encontra-se bem, com sinais clínicos e ecocardiográficos de hipertensão pulmonar discreta e sem evidências, também pelo ecocardiograma, de obstrução do tronco da artéria coronária esquerda.

We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence - also on echocardiogram - of left coronary artery trunk obstruction.

OBJETIVO: Verificar em casuísticas ambulatoriais na rede pública de saúde, em hospital terciário e na clínica privada, as características do atendimento, em cardiopatia pediátrica enfatizando o motivo de encaminhamento, o diagnóstico definitivo e a conduta adotada. MÉTODOS: Um total de 1.365 pacientes consecutivos, com idades entre 1 mês a 14 anos foram atendidos, em 1996, em 3 locais distintos: G1 (n = 562), ambulatório de referência da rede pública, oriundo de Ribeirão Preto (RP) (78%) e região (22%); G2 (n = 420), clínica privada, oriundo de RP (67%), região (25%) e outras regiões/estados (8%); G3 (n = 383), ambulatório de referência do Hospital das Clínicas da FMRP - USP, procedente de RP (26%), região (43,5%) e outras regiões/estados (30,5%). RESULTADOS: Motivo de encaminhamento: G1: sopro (71%), arritmia (8%), dor precordial (7%), dispnéia (6,5%) e motivos variados (7,5%); G2: sopro (70%), dor precordial (7%), arritmia (7%), dispnéia (4%), pós-operatório (4%) e motivos variados (8%); G3: sopro (56%), pós-operatório (24%), arritmia (4%) e motivos variados (16%). Abandono de tratamento: G1: 31%, G2: 17% e G3: 3%. Diagnóstico definitivo: G1: 346 (89%) normais e 43 (11%) anormais; G2: 268 (76%) normais e 82 (24%) anormais; G3: 22 (6%) normais e 351 (94%) anormais. Conduta: G1: alta (89%) e seguimento (11%); G2: alta (76%) e seguimento (24%); G3: alta (6%) e seguimento (94%). CONCLUSÃO: Perfil clínico diferenciado entre os três grupo. Há necessidade de intervenção nas unidades básicas de saúde para verificar condições de atendimento e oferecer treinamento cardiológico pediátrico aos pediatras. Importante determinar causas do índice de abandono no G1 e há necessidade de investir na natureza estrutural e de recursos humanos para o atendimento no ambulatório do G3.

OBJECTIVE: To verify the pediatric cardiology outpatient clinic characteristics in Ribeirão Preto (RP) city, emphasizing reasons for referral, definitive diagnosis and outcome through analysis of patients seen in 3 distinct settings. METHODS: In 1996, 1,365 consecutive patients, aged 1 month to 14 years were seen: G1 (n = 562), public pediatric cardiology outpatient clinic; G2 (n = 420), private practice; G3 (n = 383) pediatric cardiology outpatient clinic at Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto. Origin of the patients: G1: RP (78%) and region (22%); G2: RP (67%), region (25%), and other regions/states (8%); G3: RP (26%), region (43.5%), and other regions/states (30.5%). RESULTS: Reasons for referral: G1: murmur (71%), arrhythmia (8%), chest pain (7%), breathlessness (6.5%), other reasons (7.5%). G2: murmur (70%), chest pain (7%), arrhythmia (7%), breathlessness (4%), postoperative follow-up (4%), other reasons (8%). G3: murmur (56%), postoperative follow-up (24%), arrhythmia (4%), other reasons (16%). Patients lost to follow-up: G1: 31%, G2: 17%, G3: 3%. Final diagnosis: G1: 346 (89%) normal and 43 (11%) abnormal patients; G2: 268 (76%) normal and 82 (24%) abnormal patients; G3: 22 (6%) normal and 351 (94%) abnormal patients. Outcome: G1: discharge (89%), follow-up (11%); G2: discharge (76%), follow-up (24%); G3: discharge (6%), follow-up (94%). CONCLUSION: Clinical profile is different among the 3 groups (G1 and G2 are similar). Intervention in the Basic Health Units seems to be necessary to verify structural facilities and to offer basic pediatric cardiology training to pediatricians. It is important to verify the high index of patients lost to follow-up, particularly in G1. Structural and human resources are needed if adequate assistance is to be expected for the highly complex cases seen in G3. The pediatric cardiology public outpatient clinic should be maintained until resolution of cases by the pediatricians in the Basic Health Units improves.

OBJECTIVE: To access the incidence of diagnostic errors in the initial evaluation of children with cardiac murmurs. METHODS: We evaluated our 7-years of experience in a public pediatric cardiology outpatient clinic. Of 3692 patients who were sent to the hospital, 2603 presented with a heart murmur and were investigated. Patients for whom a disagreement existed between the initial and final diagnoses were divided into the following 2 groups: G1 (n=17) with an initial diagnosis of an innocent murmur and a final diagnosis of cardiopathy, and G2 (n=161) with an initial diagnosis of cardiopathy and a final diagnosis of a normal heart. RESULTS: In G1, the great majority of patients had cardiac defects with mild hemodynamic repercussions, such as small ventricular septal defect and mild pulmonary stenosis. In G2, the great majority of structural defects were interventricular communication, atrial septal defect and pulmonary valve stenosis. CONCLUSION: A global analysis demonstrated that diagnostic error in the initial evaluation of children with cardiac murmurs is real, reaching approximately 6% of cases. The majority of these misdiagnoses were in patients with an initial diagnosis of cardiopathy, which was not confirmed through later complementary examinations. Clinical cardiovascular examination is an excellent resource in the evaluation of children suspected of having cardiopathy. Immediate outpatient discharge of children with an initial diagnosis of an innocent heart murmur seems to be a suitable approach.

We describe the case of a 40-day-old female patient with a history of breathlessness since birth who was referred to our hospital for surgical correction of common arterial trunk. The invasive investigation disclosed a Fallot¢s tetralogy anatomy associated with an anomalous origin of the left pulmonary artery from the ascending aorta. Immediately after diagnosis, the patient underwent a successful total surgical correction of the defect, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk.

We report two cases of congenital atresia of the ostium of the left coronary artery. Case 1: a six-month-old infant presenting with serious cardiac insufficiency. A noninvasive diagnosis of dilated myocardiopathy was established and the clinical picture was pharmacologically compensated. When the patient was nine months of age, a hemodynamic study was performed that revealed congenital atresia of the ostium of the left coronary artery; the infant immediately underwent a successful anastomosis of the internal mammary artery with the left coronary artery. Case 2: an eleven-year-old asymptomatic boy with a history of heart murmur from the age of six months on, was refered for surgery with a diagnosis of anomalous origin of the left coronary artery from pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established during surgery. Successful surgical revascularization with the left internal mammary artery, and left ventricular aneurysmectomy were performed.

OBJECTIVE: To report the authors' experience with the anomalous origin of the left coronary artery (AOLCA) from the pulmonary trunk, emphasizing preoperative data, surgical aspects and midterm results of the follow-up. METHODS: Retrospective analysis of 11 patients operated upon at the Royal Brompton Hospital from October, 84 to April, 97. RESULTS: Nine infants had heart failure (HF) and two other children presented with dyspnea and chest pain. All had ECG changes. The echocardiogram identified the anomalous origin of the coronary artery in 7 (64%) patients and hemodynamic studies were performed in 7 patients. All infants were operated upon between the 2nd and 10th month of life. Six patients were treated with aortic reimplantation of the left coronary artery, whereas five were operated upon according to the Takeuchi technique. All patients are alive, with clear improvement of the ECG changes and ventricular function, as evaluated by echocardiography. Two patients operated upon according to the Takeuchi technique required additional surgery due to severe supravalvular pulmonary stenosis. CONCLUSION: AOLCA is a rare disease. Most patients show early signs of severe HF associated with ECG findings. Surgical therapy must be instituted early in the disease, preferentially through aortic implantation of the anomalous coronary artery, with a high possibility of success. Shortly after surgery, clinical and ECG improvement, as well as normalization of left ventricular function, should be expected.

As anastomoses sistêmico-pulmonares continuam sendo um importante procedimento no tratamento e na preparação dos cardiópatas cianóticos, com hipoplasia das artérias pulmonares, para a correção total. Todas têm vantagens e desvantagens e os resultados dependerão de idade e peso dos pacientes e da complexidade da cardiopatia. O presente estudo relata a experiência com sete casos de anastomoses mamária-artéria pulmonar, realizadas através de toracotomia direita (5) e esquerda (2), em pacientes portadores de tetralogía de Fallot (quatro femininos e três masculinos). A idade variou de dois a 63 meses (m = 18,4), com peso médio de 7,9 kg. Seis apresentavam graus variados de hipoplasia pulmonar. Houve dois óbitos pós-operatórios (1º e 2º dias), devidos a trombose da artéria mamária, no local de seu clampleamento. Dois pacientes foram submetidos a correção total (um mês e três anos após): no 1º (menina de 24 meses, 10 kg de peso), a anastomose foi feita pelas más condições gerais, apesar do bom tamanho das pulmonares. No 2º (menino de 15 meses, 8,1 kg, no qual uma operação de Blalock-Taussig clássica foi feita e trombosou no 3º mês de vida), foi possível observar o progressivo desenvolvimento das artérias pulmonares, por estudo hemodinâmico. Em ambos, o funcionamento da anastomose era perfeito e a correção foi bem sucedida. Três outros pacientes aguardam o momento oportuno para a correção final. Apesar do reduzido número e do curto tempo de observação, acredita-se que a anastomose mamáriapulmonar possa ser outra opção paliativa para os portadores de cardiopatias congênitas cianogênicas, com hipoplasia das artérias pulmonares e que os melhores resultados devam ser obtidos nos pacientes maiores, fora de situações de emergência e com pequenas artérias pulmonares.

The systemic-pulmonary shunts are an important procedure to treat and prepare, for the final correction, cyanotic patients with hypoplastic pulmonary arteries. All of them have advantages and disadvantages, and the results will depend on the age and weight of the patients and the complexity of their heart disease. The present study reports seven internal mammary-pulmonary anastomosis, made through right (2) or left (5) thoracotomies, in patients with Tetralogy of Fallot and hypoplastic pulmonary arteries, from 2 to 63 months of age (m = 18,4) and mean weight of 7.9 kg. There were two deaths (1st and 2nd days), due to thrombosis of the internal mammary, where it was clamped. Two patients went to total correction (1 month and 3 years later). In the first case, the shunt was made due to the general conditions of the child, despite the good size of the pulmonary arteries. In the second case (a 15 months old boy, in whom a classical Blalock-Taussig shunt was made and thrombosed in the 3rd month of life) was possible to follow the progressive enlargement of the pulmonary arteries. In both the shunts were well functioning and the total correction was successful. Three patients are waiting for their best time for final procedures. Despite the small number and the short period of observations, the authors do believe that the internal mammary-pulmonary artery anastomisis is palliative option for these patients. The best results are obtained among the eldest children that are not in an emergency situation and not involving small pulmonary arteries.