RESUMO
OBJECTIVE: To determine whether pectus excavatum (PE) results in cardiopulmonary abnormalities, and whether surgical repair results in improvement. METHODS: We performed pulmonary function testing and incremental exercise testing in 36 adolescents with PE (aged 16 +/- 3 (SD) years) and 10 age-matched, healthy control subjects. Fifteen PE subjects were reexamined postoperatively, as were six control subjects. RESULTS: Preoperatively, PE subjects had a significantly lower forced vital capacity than control subjects had (81% +/- 14% vs 98% +/- 9% of the predicted value; p < 0.001). Chest computed tomography ratios of internal transverse to antero-posterior diameters correlated inversely with total lung capacity (r = 0.56; p < 0.01). Fifty-eight percent of PE subjects had subjective complaints of exercise limitation. PE subjects exercised at a workload similar to that of control subjects. Maximal heart rate and O2 pulse did not differ between the two groups. Respiratory measurements during exercise were similar between the two groups. Respiratory measurements during exercise were similar between the two groups. Postoperatively there was no change in forced vital capacity (as a percentage of the predicted value). The PE subjects exercised for a slightly longer period and had a slightly higher O2 pulse, whereas control subjects showed no change. CONCLUSION: Some subjects with PE have mild restrictive lung disease, which is not affected by surgical repair. Postoperatively they have a slight increase in exercise tolerance and O2 pulse, which suggests improved cardiac function during exercise. However, the clinical implications of this modest improvement are unclear.
Assuntos
Tórax em Funil/cirurgia , Respiração/fisiologia , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Teste de Esforço , Feminino , Seguimentos , Tórax em Funil/fisiopatologia , Humanos , Masculino , Período Pós-Operatório , Testes de Função Respiratória , Resultado do TratamentoRESUMO
We reviewed the records of 28 patients with Marfan syndrome and 30 age-matched control patients with presumed isolated pectus excavatum to determine the outcome of surgical repair of the pectus deformity in Marfan syndrome. One third of the patients with Marfan syndrome underwent repair of the pectus excavatum before diagnosis. Of the 30 patients with "isolated" pectus excavatum, 17 had findings by history or physical examination, such as mitral valve prolapse, scoliosis, or a relative with pectus excavatum, suggestive of an underlying disorder of connective tissue. Pectus excavatum of more than moderate severity recurred in 11 of 28 patients with Marfan syndrome and was associated with young age at initial surgery and lack of temporary internal stabilization of the chest after surgery. Only two of the control patients had recurrence of the defect; one of these patients had findings suggestive of an underlying heritable disorder of connective tissue. We conclude that pectus excavatum may indicate the presence of an underlying heritable disorder of connective tissue such as the Marfan syndrome. In patients with Marfan syndrome, and possibly other inherited connective tissue disorders, surgical repair should be delayed if possible until skeletal maturity is nearly complete and should employ internal stabilization.
Assuntos
Tórax em Funil/cirurgia , Síndrome de Marfan/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Lactente , Síndrome de Marfan/complicações , Prontuários Médicos , Período Pós-Operatório , Prognóstico , RecidivaRESUMO
Acute renal failure occurred in association with cardiac surgery in 20 of 248 infants (8%). Hypotension, poor tissue perfusion, and hypoglycemia were the most important factors recognized in the pathogenesis and outcome of the ARF. However, many infants were extremely ill preoperatively. The most frequent operative procedures performed in the 20 patients were open-heart surgery with total correction under deep hypothermia and repair of coarctation of the aorta. Thirteen of the 20 infants with ARF died. The combination of a major operative procedure, cardiac failure, hypoglycemia, hypotension, and compromised renal function imposes important constraints in the treatment of hyperkalemia, hypoglycemia, correction of acid-base distrubances, and the administration of fluids.