Heart transplantation in neonates and children. Intermediate-term results

OBJECTIVE: To assess intermediate-term outcome in children who have undergone orthotopic heart transplantation. METHODS: We carried out a longitudinal and prospective study between October '92 and June '99 comprising 20 patients with ages ranging from 12 days to 7 years (mean of 2.8 years). We employed a double immunosuppression protocol with cyclosporine and azathioprine and induction therapy with polyclonal antithymocyte serum. Survival and complications resulting from the immunosuppression protocol were analyzed. RESULTS:The double immunosuppression protocol and the induction therapy with polyclonal antithymocyte serum resulted in an actuarial survival curve of 90% and 78.2% at 1 and 6 years, respectively, with a mean follow-up period of 3.6 years. One patient died due to acute rejection 40 days after transplantation; another patient died 2 years after transplantation due to lymphoproliferative disorder; a third patient died because of primary failure of the graft; and a fourth patient died due to bronchopneumonia. The major complications were as follows: acute rejection, infection, nephrotoxicity, and systemic hypertension. The means of rejection and infection episodes per patient were 2.9 and 3.4, respectively. After one year of transplantation, a slight reduction in the creatinine clearance and systemic hypertension were observed in 7 (38.9%) patients. CONCLUSION: Heart transplantation made life possible for those patients with complex congenital heart diseases and cardiomyopathies in refractory congestive heart failure constituting a therapeutical option for this group of patients in the terminal phase.

Mediastinitis after cardiac transplantation

OBJECTIVE: Assessment of incidence and behavior of mediastinitis after cardiac transplantation. METHODS: From 1985 to 1999, 214 cardiac transplantations were performed, 12 (5.6%) of the transplanted patients developed confirmed mediastinitis. Patient's ages ranged from 42 to 66 years (mean of 52.3±10.0 years) and 10 (83.3%) patients were males. Seven (58.3%) patients showed sternal stability on palpation, 4 (33.3%) patients had pleural empyema, and 2 (16.7%) patients did not show purulent secretion draining through the wound. RESULTS: Staphylococcus aureus was the infectious agent identified in the wound secretion or in the mediastinum, or both, in 8 (66.7%) patients. Staphylococcus epidermidis was identified in 2 (16.7%) patients, Enterococcus faecalis in 1 (8.3%) patient, and the cause of mediastinitis could not be determined in 1 (8.3%) patient. Surgical treatment was performed on an emergency basis, and the extension of the débridement varied with local conditions. In 2 (16.7%) patients, we chose to leave the surgical wound open and performed daily dressings with granulated sugar. Total sternal resection was performed in only 1 (8.3%) patient. Out of this series, 5 (41.7%) patients died, and the causes of death were related to the infection. Autopsy revealed persistence of mediastinitis in 1 (8.3%) patient. CONCLUSION: Promptness in diagnosing mediastinitis and precocious surgical drainage have changed the natural evolution of this disease. Nevertheless, observance of the basic precepts of prophylaxis of infection is still the best way to treat mediastinitis.

Outcome of acute renal failure associated with cardiac surgery in infants

OBJECTIVE: To analyze the impact of acute renal failure (ARF) on the evolution of infants undergoing cardiac surgery. METHODS: We assessed 15 infants undergoing cardiac surgery who developed (ARF). Their demographic, clinical and surgical data, and evolution were analyzed. RESULTS: Their mean age was 4.4±4.0 months (8 days to 24 months). Twelve infants were males, and 4 patients already had ARF at surgery. The primary cause of ARF was immediate acute cardiac dysfunction in 10 infants, cardiac dysfunction associated with sepsis in 2 infants, and isolated sepsis in 3 infants. All children depended on mechanical ventilation during their postoperative period, 14 infants used vasoactive drugs, and 11 had an infectious process associated with ARF. Thirteen infants required dialytic treatment. Eleven infants developed oluguric ARF, and all had to undergo peritoneal dialysis; of the 4 patients with non-oliguric, 2 required dialysis, the main indication being hypervolemia. Of these 13 dialyzed infants, 4 died in the first 24 hours because of the severity of the underlying cardiac disease (mean urea level of 49±20 mg/dl). The mortality rate for the entire group was 60% , and it was higher among the patients with oliguria ARF (73% vs 25%, p<0.001). The cause of death was acute cardiac dysfunction in 6 infants (early type-1ARF) and sepsis in the 3 remaining infants (late type-2 ARF). CONCLUSION: The mortality rate of ARF associated with cardiac surgery in infants was hight, being higher among children with oliguria; peritoneal dialysis was indicated due to clinically uncontrolled hypervolemia and not to the uremic hypercatabolic state.

Analysis of the risk factors for allograft vasculopathy in asymptomatic patients after cardiac transplantation

OBJECTIVE: To study the influence of immune and nonimmune risk factors on the development of allograft vasculopathy after cardiac transplantation. METHODS: We studied 39 patients with a mean age of 46±12 years. The following variables were analyzed: weight (kg), body mass index (kg/m²), donor's age and sex, rejection episodes in the first and second years after transplantation, systolic and diastolic blood pressures (mmHg), total cholesterol and fractions (mg/dL), triglycerides (mg/dL), diabetes, and cytomegalovirus infection. The presence of allograft vasculopathy was established through coronary angiography. RESULTS: Allograft vasculopathy was observed in 15 (38%) patients. No statistically significant difference was observed between the two groups in regard to hypertension, cytomegalovirus infection, diabetes, donor's sex and age, rejection episodes in the first and second years after transplantation, and cholesterol levels. We observed a tendency toward higher levels of triglycerides in the group with disease. Univariate and multivariate analyses showed statistically significant differences between the two groups when we analyzed the body mass index (24.53±4.3 versus 28.11±4.6; p=0.019). CONCLUSION: Body mass index was an important marker of allograft vasculopathy in the population studied.

Pregnancy after cardiac transplantation. Report of one case and review

A 14-year-old female patient became pregnant 6 years after heart transplantation. The pregnancy evolved uneventfully, and the newborn infant was healthy. Five months after delivery, the mother was in good condition with preserved ventricular function, and the baby had normal neuro-psychomotor development. Even though the case reported here was a success, pregnancy following cardiac transplantation is considered a high-risk condition and remains contraindicated.

Noninvasive diagnosis of allograft vascular disease after heart transplantation

OBJECTIVE: To determine the predictive values of noninvasive tests for the detection of allograft vascular disease. METHODS: We studied 39 patients with mean ages of 48±13 years and a follow-up period of 86±13 months. The diagnosis of allograft vascular disease was made by cine-coronary arteriography, and it was considered as positive if lesions existed that caused > or = 50% obstruction of the lumen. Patients underwent 24h Holter monitoring, thallium scintigraphy, a treadmill stress test, and dobutamine stress echocardiography. Sensitivity, specificity, and positive and negative predictive values were determined in percentages for each method, as compared with the cine-coronary arteriography results. RESULTS: Allograft vascular disease was found in 15 (38%) patients. The Holter test showed 15.4% sensitivity, 95.5% specificity. For the treadmill stress test, sensitivity was 10%, specificity was 100%. When thallium scintigraphy was used, sensitivity was 40%, specificity 95.8%. On echocardiography with dobutamine, we found a 63.6% sensitivity, 91.3% specificity. When the dobutamine echocardiogram was associated with scintigraphy, sensitivity was 71.4%, specificity was 87%. CONCLUSION: In this group of patients, the combination of two noninvasive methods (dobutamine echocardiography and thallium scintigraphy) may be a good alternative for the detection of allograft vascular disease in asymptomatic patients with normal ventricular function.

Histoplasmosis as a late infectious complication following heart transplantation in a patient with Chagas' disease

Infectious complications following heart transplantation are an important cause of morbidity and mortality. Generally, bacterial infections are predominant; however, fungal infections can be responsible for up to 25% of infectious events. We report the case of a patient who presented with histoplasmosis as an infectious complication five years after heart transplantation due to a chagasic cardiopathy. This association has rarely been reported in the international literature.

Uhl's anomaly. Differential diagnosis and indication for cardiac transplantation in an infant

We report the case of an 8-month-old female infant with Uhl's anomaly, who underwent successful cardiac transplantation. The clinical findings, complementary laboratory tests, anatomic findings, and differential diagnosis of the anomaly are discussed.

Effects of high glucose concentrations on the endothelial function of the renal microcirculation of rabbits

OBJECTIVE: To assess the acute effects of high glucose concentrations on vascular reactivity in the isolated non diabetic rabbit kidney. METHODS: Rabbits were anaesthetized for isolation of the kidneys. Renal arteries and veins were cannulated for perfusion with Krebs-Henselleit solution and measurement of perfusion pressure. After 3 hours of perfusion with glucose 5,5 mM (control ) and 15 mM, the circulation was submitted to sub maximal precontraction (80% of maximal response) trough continuous infusion of noradrenaline 10 mM. Vascular reactivity was then assessed trough dose-responses curves with endothelium-dependent (acetylcholine) and independent (sodium nitroprusside) vasodilators. The influence of hyperosmolarity was analyzed with perfusion with mannitol 15mM. RESULTS: A significant reduction in the endothelium-dependent vasodilation in glucose 15mM group was observed compared to that in control, but there was no difference in endothelium-independent vasodilation. After perfusion with mannitol 15 mM, a less expressive reduction in endothelium-dependent vasodilation was observed, only reaching significance in regard to the greatest dose of acetylcholine. CONCLUSION: High levels of glucose similar to those found in diabetic patients in the postprandial period can cause significant acute changes in renal vascular reactivity rabbits. In diabetic patients these effects may also occur and contribute to diabetes vascular disease.

Insuficiência renal aguda após cirurgia de revascularização miocárdica com circulação extracorpórea: incidência, fatores de risco e mortalidade

OBJETIVO: Identificar a incidência, fatores de risco e mortalidade de insuficiência renal aguda (IRA), em pacientes submetidos à cirurgia para revascularização miocárdica com circulação extracorpórea. MÉTODOS: Estudados prospectivamente todos os pacientes submetidos a cirurgia de revascularização miocárdica e as variáveis clínicas e laboratoriais analisadas através de métodos uni e multivariado (regressão logística). RESULTADOS: Insuficiência renal aguda ocorreu em 16,1% dos 223 pacientes estudados, diálise foi necessária em 4,9% dos pacientes. Os fatores de risco associados à IRA na análise univariada foram: idade > 63 anos OR 3,6 (95% IC=1,6 a 8,3), creatinina sérica pré-operatória > 1,2 mg/dl OR 5,9 (95% IC=2,4 a 14,6), duração da circulação extracorpórea > 90 min OR 2,1 (95% IC=1,0 a 4,4), uso de balão intra-aórtico OR 2,6 (95% IC=1,2 a 5,5); necessidade de drogas inotrópicas OR 4,4 (95% IC=1,9 a 10,2) e, na análise multivariada, foram fatores independentes associados à IRA idade > 63 anos OR 3,0 (95% IC=1,3 a 7,2), creatinina sérica pré-operatória > 1,2 mg/dl OR 4,3 (95% IC=1,6 a 11,4), necessidade de drogas inotrópicas OR 3,2 (95% IC=1,3 a 8,0). A mortalidade nos pacientes com IRA foi de 25,0 % em comparação com 1,1% entre os sem IRA e 63,6% entre os que necessitaram de diálise. CONCLUSÃO: Insuficiência renal aguda em cirurgia de revascularização miocárdica é uma complicação freqüente e está associada à alta mortalidade. Sendo fatores de risco independentes: idade, insuficiência renal prévia e necessidade de drogas inotrópicas.

Doença cardiovascular e fatores de risco cardiovascular em candidatos a transplante renal

OBJETIVO: Determinar a prevalência de doença cardiovascular (DCV) e de fatores de risco tradicionais em portadores de insuficiência renal crônica em avaliação para inclusão em lista para transplante renal. MÉTODOS: Foram submetidos à avaliação clínica e exames complementares 195 pacientes com insuficiência renal crônica dialítica e comparados a grupo de 334 hipertensos pareados por idade. As equações de Framingham foram usadas para o cálculo do risco absoluto (RA); o risco relativo (RR) foi calculado tendo como referência o risco absoluto da coorte de baixo risco de Framingham. RESULTADOS: Do total, 37% apresentaram algum tipo de doença cardiovascular na avaliação inicial, sendo que arteriopatia obstrutiva (23%) foi a mais prevalente. Excluídos os pacientes com doença cardiovascular, em relação aos fatores de risco tradicionais, houve diferença significativa quanto à pressão arterial sistólica e colesterol total (maiores no grupo de hipertensos) e às prevalências de homens, diabetes e tabagismo, maiores no grupo de insuficiência renal crônica, que apresentou maior grau de hipertrofia ventricular esquerda, menor pressão arterial diastólica e menor prevalência de história familiar de doença cardiovascular e obesidade. O risco relativo para doença cardiovascular dos pacientes com insuficiência renal crônica foi mais elevado em relação à população controle de Framingham porém não diferiu da observada no grupo de hipertensos. CONCLUSÃO: Em candidatos a transplante renal é significativa a prevalência de doença cardiovascular e de fatores de risco tradicionais; as equações de Framingham não quantificam adequadamente o risco cardiovascular real e outros fatores de risco específicos desta população devem contribuir para o maior risco cardiovascular.

Insuficiência renal aguda após uso de imunoglobulina intravenosa para tratamento de miocardite

Mulher de 60 anos com quadro de insuficiência cardíaca classe funcional III/IV (NYHA) de etiologia imuno-mediada (miocardite autoimune). Após tentativas terapêuticas sem sucesso, foi utilizada imunoglobulina intravenosa, deteriorando a função renal, complicação rara desta terapia. Após hemodiálise a paciente recuperou a função renal e ocorreu melhora da insuficiência cardíaca crônica para classe funcional I.

Endomiocardiofibrose biventricular associada à amiloidose renal

A endomiocardiofibrose (EMF) é uma cardiomiopatia restritiva, caracterizada por envolvimento fibrótico do endocárdio e miocárdio adjacente, e disfunção diastólica, causada por alteração da distensibilidade, dificultando o enchimento ventricular adequado, estando a função sistólica preservada. Clinicamente, apresenta-se como insuficiência cardíaca, mas, para o diagnóstico etiológico correto, são necessárias perspicácia semiológica, suspeita e investigação clínica. Relatamos o caso de uma paciente portadora de endomiocardiofibrose biventricular associada à amiloidose renal.