Detection of an aneurysm of the vein of Galen following signs of cardiac overload in a 22-week old fetus.

OBJECTIVES: To present early prenatal diagnosis of an aneurysm of the Vein of Galen by detecting signs of cardiac overload in a 22-week old fetus. CASE: We report on a 22-week old fetus presenting with dilation of the heart, tricuspid and mitral valve regurgitation, reversed flow in the aortic arch and dilated neck vessels. Evaluation of the fetal brain revealed a dilated left ventricle, signs of brain hemorrhage and an anechoic structure between the two hemispheres. METHODS: By using the conventional Doppler technique, an intracerebral fistula and dilated vein of Galen was visualized. The complex vascular structure was demonstrated using 3-D color power angiography (3-D CPA). To our knowledge, this is the earliest diagnosis of this vascular malformation. CONCLUSION: Although signs of cardiac overload rarely appear before the third trimester in cases of arteriovenous fistulae, it was shown that these signs could lead to the correct diagnosis of an aneurysm of the vein of Galen. Additionally, we show that 3-D CPA is a promising technique to visualize complex vascular structures and make them easier to understand.

Permanent pacemaker implantation in a 1,445 g preterm neonate on the first day of life.

Here, we present a case involving a very low-birthweight baby (1445 g) born prematurely after 30 weeks of gestation with congenital complete heart block and low-output failure. The newborn was successfully treated by implantation of an epimyocardial pacemaker on her first day of life.

Successful management of a B-type cardiac allograft into an O-type man with 3(1/2)-year clinical follow-up.

BACKGROUND: In May 1997, a 19-year-old male patient of histo-blood group type O suffering from congenital end-stage heart failure accidentally received a cardiac allograft of type B and is still alive in fair condition. METHODS: In addition to conventional immunosuppressive therapy, plasma exchange (PEX), extracorporeal immunoabsorption (EIA), intravenous immunoglobulins (IVIG), and C1 inhibitor were used. RESULTS: Such treatment successfully reduced both IgM and IgG anti-B levels and complement hyperactivity and allowed to reach the state of accommodation without obvious signs of rejection. The patient has been surviving for 42 months; retransplantation with an O-type heart remained unnecessary. CONCLUSION: Humoral rejection has been avoided in this patient, with PEX, EIA, IVIG, and C1 inhibitor substantially contributing to this success. With future availability of such combined therapies, preferably before transplantation, vascular rejection events caused by preformed antibodies and complement (ABO mismatch or anti-HLA) could be prevented or treated.

Correçäo cirúrgica precoce da artéria coronária esquerda com origem em tronco pulmonar: evoluçäo tardia / Early surgical correction of the left coronary artery with origin at the pulmonary trunk. Late development

Este relato mostra em seus dois casos a acentuada e progressiva melhora da contratilidade ventricular esquerda no pós-operatório tardio de paciente submetidos à correçäo cirúrgica da origem anômala da artéria coronária esquerda do tronco pulmonar. Lactentes, um de sexo masculino e outro de sexo feminino, desenvolveram quadro de insuficiência cardíaca congestiva, näo controlada clinicamente. O diagnóstico em ambos os casos foi confirmado por cineangiocoronariografia. Foram submetidos à coronária esquerda do tronco pulmonar para a aorta. Após nove e sete anos, respectivamente, encontram-se livres de sintomas, com acentuada recuperaçäo da contratilidade ventricular, sem déficit pôndero-estatural e em excelentes condiçöes clínicas. O tratamento cirúrgico precoce destes pacientes, mesmo em condiçöes clínicas desfavoráveis, é viável e pode alterar a história natural desta doença propiciando uma melhora acentuada e progressiva da contratilidade ventricular

The report of these two cases show the accentuated and progressive increase in the ventricular contractility in the late postoperative period in patients who underwent surgical correction of left coronary artery with origin at the pulmonary trunk. Male and female infants, both developed congestive heart failure not controlled by medical treatment. The diagnosis was made by EKG and confirmed by coronaryangiogram. The were surgically treated by translocation of the left coronary artery from the pulmonary artery to the aorta. Nine and seven years later, respectively both patients are free of symptoms with marked recovery of the left ventricle function without alteration in height and weight and in excellent clinical conditions. The early surgical treatment in these cases, even with unfavourable clinical status is the only way to change the natural history of this patology, with an increase of the ventricular contractility.