congenital cardiopathy

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Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect

OBJECTIVE: To determine the long-term natural history of patients with mild pulmonary valve stenosis METHODS: Throughout a 24-year evolutive period (1980 a 2004), 83 patients with pulmonary valve stenosis were evaluated, with 29 of them presenting a mild form of the defect. Of these 29, 13 patients had a long-term follow up. The mean age at the first and last follow-up visits was 34 months (1 month to 15 years) and 10.5 years (3 to 24 years), respectively. In addition to evolutive elements, those regarding clinical, electrocardiographic and echocardiographic aspects were also assessed. RESULTS: All patients remained assymptomatic throughout the study. Regarding the pulmonary valve pressure gradient, 5 (38.4%) presented a decrease, 3 (23%) remained unchanged, 4 (30.7%) presented elimination and 1 (7.6%) presented accentuation to a moderate degree of involvement. The mean initial and final gradient was 24.2 mmHg (15-30) and 13.6 mmHg (0-54), respectively. The initial gradients of the 4 patients who achieved spontaneous cure were 30, 19, 30 and 20 mmHg. The systolic murmur and right ventricular overload had a direct correlation to the gradient pressure degree, subdivided at each 10 mmHg. CONCLUSION: The spontaneous cure of the mild pulmonary valve stenosis is possible to achieve, similarly to other acyanogenic anomalies.

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Interventricular septal defects percutaneous occlusion: initial experiment

Francisco José Araújo Chamié de Queiróz et al. Raul Ivo Rossi Filho Sérgio Ramos César Esteves … Daniel Silva Chamié de Queiróz Paulo Renato Machado João Carlos Tress Stella Suzana Horowitz Helder Paupério Rosaura Victer

OBJECTIVE: To assess the initial experiment with percutaneous closure of interventricular septal defects (IVSD), especially perimembranous IVSD (PMIVSD) with the new Amplatzer prosthesis. METHODS: Eleven patients were submitted to the procedure. Six of them had perimembranous IVSD (PMIVSD) and five with muscular IVSD (MIVSD). Two showed repeated respiratory tract infections and had low ponderal gain. One of them showed a previous history of infectious endocarditis. The others were asymptomatic and were selected through transthoracic echocardiography (TTE). RESULTS: In MIVSD group (n=5), a patient had apical IVSD, two had medioseptal defects and two patients showed multiple defects. In this group, an interatrial septal defects (IASD) (Amplatzer Septal Occluder®) and five prostheses for MIVSD (Amplatzer VSD-MUSC Occluder®), and one patient received two devices. All implants were well-succeeded and went by without complications. In PMIVSD group (n=6), two patients had membranous septal aneurysms. In five, we identified two orifices with selective angiography. We used arterial canal Amplatzer prostheses (ACP) (Amplatzer Duct Occluder®) in 1 patient and specific prostheses for PMIVSD in the others (Amplatzer VSD-MEMB Occluder®). A perimembranous occluder migrated immediately after released, being removed from the descending aorta. Another patient showed total atrioventricular block (TAVB), which reversed after corticotherapy. CONCLUSION: The procedure is technically complex and must be performed only in specialized centers. Despite of that, it showed safe and efficient in the selected cases, and it can be provided as an alternative to traditional surgical treatment.

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Original Article

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

Fernando Amaral et al. João A. Granzotti

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.

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Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

Gabriel Lorier et al. Renato A.K. Kalil Gustavo R. Hoppen Christiano Barcellos … Nicolas Teleo Abud Homsi Netto Javier Gonzalez Paulo R. L. Prates Paulo R. Prates João R. M. Sant'Anna Ivo A. Nesralla

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67±3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases), stenosis 28.6% (6 cases), and double lesion 14.3% (3 cases). The perfusion 43.10±9.50min, and ischemia time were 29.40±10.50min. The average clinical follow-up in mitral insufficiency was 41.52±53.61 months. In the stenosis group (4 patients) was 46.39±32.02 months, and in the double lesion group (3 patients), 39.41±37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17±39.51 months, stenosis 42.61±30.59 months, and in the double lesion 39.41±37.51 months. RESULTS: Operative mortality was 9.5% (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3%) patients were asymptomatic (p=0.04). The majorit y with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

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Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect

Interventricular septal defects percutaneous occlusion: initial experiment

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

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

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