Outcome of surgical repair of multiple muscular ventricular septal defects (Swiss cheese septum) has improved. Transatrial re-endocardialization strategy enables early complete or nearly complete obliteration of multiple muscular ventricular septal defects with minimal residual lesions (shunt, ventricular dysfunction). Long cardiopulmonary bypass duration is well tolerated. The incidence of permanent heart block has improved. Early echocardiographic and clinical outcomes are promising.
An anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (interarterial coronary artery [IACA]) is a rare congenital anomaly associated with sudden cardiac death. The cardiac and surgical databases at a single pediatric institution were reviewed for a description of the clinical profiles and associated risk factors of this coronary anomaly. From 1994 to 2006, IACA was diagnosed for 31 patients with a median age of 6.2 years (range, birth to 16 years). The symptoms for 6 (19%) of the 10 patients (32%) presenting with symptoms were deemed to be cardiac in origin. The symptoms for the remaining 21 patients (68%) were incidental findings. Of the 31 patients, 29 (94%) had normal resting electrocardiograms (ECG). A total of 17 patients underwent dobutamine stress echo. None had wall motion abnormalities, but two had ECG changes indicating ischemia, and 4 had abnormal coronary flow, as detected by Doppler echocardiography. Seven patients, with either acute symptoms or testing suggestive of ischemia, underwent surgery. All seven had an interarterial left main coronary artery. There was one cardiac-related death. No sudden death was found in either the surgery or nonsurgery group during the mean follow-up period of 23 and 58 months, respectively. Whether surgical intervention modifies the natural history of the anomaly or not remains to be determined.
Human parvovirus B19 (B19 virus) infection is known to induce aplastic crisis in patients with hemolytic anemia. In healthy subjects, B19 infection may sometimes cause mild pancytopenia, but these changes are transient and recovery is spontaneous. We report the first case of aplastic anemia in a previously healthy boy without any underlying diseases, following asymptomatic infection with the B19 virus. Laboratory examination initially showed thrombocytopenia, mild leukopenia in the peripheral blood, and severe hypoplastic bone marrow. Since pancytopenia developed and worsened progressively, immunosuppressive therapy was given, resulting in a complete remission. Despite the lack of an infectious prodrome, serological and histological analysis revealed an underlying infection with the B19 virus. Thus, B19 virus infection must be considered one of the causes of aplastic anemia in patients without underlying hemolytic anemia and an apparent episode of the viral infection.
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