Joäo A Granzotti

Incidence of heart defects in Down syndrome

Eighty-six patients with Down Syndrome were studied with the main purpose of quantifying the incidence of congenital heart defects and the risk of occurrence according to the mothers age. Thirty-eight patients had the cariotypes determined, 35 of them having trissomy of chromosome 21 and translocation in 3 cases. Congenital heart disease was found in 44 (51%) of the patients, the most common one being ventricular septal defect. An important incidence of Fallots tetralogy was also found (20%). These 86 children were submitted to 41 surgical procedures, most of them on the cardiovascular system. The maternal mean age was 33 -/+ 8.6 years and the estimated risk of Down Syndrome was 1/590, a lower value than the one reported in other studies.

Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients

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 referred 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.

Compressão extrínseca da artéria coronária esquerda por dilatação aneurismática do tronco pulmonar em adolescente: involução após oclusão cirúrgica de comunicação interatrial seio venoso e plastia redutora do tronco pulmonar

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.

Cardiopatia congênita no adulto: perfil clínico ambulatorial no Hospital das Clínicas de Ribeirão Preto

BACKGROUND: Service experiences for adults with congenital heart disease have not been reported in our country. OBJECTIVE: To describe the basic clinical profile of adults with congenital heart disease in an outpatient tertiary care center. METHODS: We compiled data on age, gender, place of residence, primary diagnosis, and secondary diagnoses of 413 patients treated for seven years. RESULTS: G1 (untreated): 195 patients, 51% women, 57% between 14 and 30 years, 80% living in the region. The most frequent heart diseases were ventricular septal defect (VSD) (31%), atrial septal defect (ASD) (29%), and pulmonary stenosis (7%). The predominant secondary diagnoses were hypertension (9%) and arrhythmias (5%). G2 (treated): 218 patients, 56% women, 57% between 14 and 30 years, 81% living in the region. The most frequently treated heart diseases were: ASD (36%), tetralogy of Fallot (14%), coarctation of the aorta (12%), and VSD (11%). Sixty-nine (32%) patients were operated on for congenital heart diseases in adulthood. Sixteen (7%) underwent an interventional catheterization. The predominant secondary diagnoses were hypertension (18%) and arrhythmias (8%). CONCLUSION: In the study, most patients were treated invasively, all of them were residents in the region, and most of them were under 40 years of age. Defects such as ASD, VSD, and pulmonary stenosis predominated in the untreated group, whereas in the treated group, most patients had undergone surgical correction of ASD, tetralogy of Fallot, aortic coarctation, and VSD. Hypertension and arrhythmias were relevant in both groups, and a large variety of other comorbidities were also observed.

Anomalous origin of the left pulmonary artery from the ascending aorta: successful surgical correction in an infant with fallot's tetralogy

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 cent 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.

Cardiologic evaluation of children with suspected heart disease: experience of a public outpatient clinic in Brazil

CONTEXT During initial evaluation of children on an outpatient basis, the index of suspected heart disease may be high, particularly if we consider that innocent murmur occurs in about 50% of the pediatric population. This is the most common cause of referral to the pediatric cardiologist. OBJECTIVE To report on the experience of a public outpatient clinic in the southeastern region of Brazil. DESIGN Retrospective analysis of all patients submitted to cardiologic evaluation within a 39 month period. SETTING Public pediatric cardiology outpatient clinic. PARTICIPANTS 2675 consecutive children aged</=15 years referred from the local and regional basic health units due to suspected heart disease. MAIN MEASUREMENTS Reason for referral, diagnostic investigation, final diagnosis based on the reason for referral, therapeutic procedures. RESULTS The main reasons for referral were: murmur (70%), precordial pain (9%), suspicion of arrhythmia (9%) and breathlessness (5%). Of the total number, 695 cases (26%) did not complete the investigation and were not included in the analysis. A final diagnosis was obtained based on the reason for referral and the main conclusions were: l) a high incidence of normality was found: murmur (83%), pain (98%), arrhythmia (97%) and breathless (94%); 2) heart disease was unlikely, based on other referral reasons; 3) 14% of the children were considered abnormal and 1% needed therapeutical procedures. CONCLUSIONS The establishment of a pediatric cardiology outpatient clinic within the public health service in the region seems to be justifiable, due to the high current demand. The low global incidence of heart disease, with a high prevalence of children with innocent murmur, discloses the need for a specific training program in cardiology for pediatricians.

Initial diagnostic errors in children suspected of having heart disease: prevalence and long-term consequences

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.

Profile of pediatric outpatient in cardiology clinics in the city of Ribeirão Preto

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.

Congenital rubella syndrome and the occurrence of congenital heart disease

Aiming to document the incidence and type of associated congenital heart disease, 20 children affected with the congenital rubella syndrome have been evaluated during a 5 year period, starting 1988. Congenital heart disease was detected in 45% of the cases. Persistent ductus arteriosus was the most frequent finding, followed by ventricular and atrial septal defects. Hearing disturbances occurred in 83% of the 12 cases studied. Ophthalmic lesions appeared in 69% of the 13 cases analysed, congenital cataracts being the most frequent one. An association of 66% was found between ophthalmic and heart lesions. Regarding the nutritional index, 70% of the cases were situated between the 25th and 50th percentile. The relevant incidence of congenital defects and the documentation of an important number of cases in a short period of time point out to a significant prevalence of the syndrome with imperative need improve the vaccination programs.

Cardiac evaluation in children with suspected heart disease. Preliminary results in 2000 patients

Two thousand consecutive children have been evaluated for suspected heart disease in a 27-month period. The main reasons for referral were: murmur (70%), precordial pain (9.5%), suspicion of arrhythmia (8.5%) and breathless (5%). Five hundred and six (25%) cases did not complete the investigation and the results were not computed. A final diagnosis was obtained based on the reason for referral and the main conclusions were: 1) a high incidence of normality was found: murmur (83%), pain (98%), arrhythmia (97%) and breathless (94%); 2) heart disease is unlikely when other referral reasons were analyzed; 3) 14% of the children were considered abnormal and the necessity of therapeutic procedures was 0.8%. A pediatric cardiology outpatient clinic in a public setting seems to be justifiable in the region, due to the high current demand.