Effectiveness and safety of balloon dilation of native aortic coarctation in premature neonates weighing < or = 2,500 grams.

Six neonates weighing < or = 2,500 g with native coarctation of the aorta underwent balloon dilation. Of the 6 neonates, 4 were female and 2 were male, with a mean age of 14 days (range 9 to 20) and a mean weight of 1,900 g (range 790 to 2,500). The procedure was acutely successful in all 6 patients; the peak gradient decreased from 38 + or - 19 mm Hg to 11 + or - 3 mm Hg. The diameter increased from 1.5 + or - 0.6 mm to 3.6 + or - 0.7 mm. Of the 6 patients, 3 had required no additional intervention at a mean of 42 months after the initial dilation, and 3 had developed restenosis a mean of 2.4 months after the initial dilation and underwent successful redilation. Of the latter 3 patients, 2 developed restenosis and underwent surgical repair 37 and 68 days after the second dilation, and 1 of these patients developed recoarctation after surgery that was treated successfully with balloon dilation 54 days after the end-to-end repair. In conclusion, premature neonates weighing < or = 2,500 g with coarctation of the aorta appear to respond acutely to balloon dilation. Some patients will have a successful long-term result after a single balloon dilation procedure. However, restenosis is common and tends to develop rapidly.

Dextrocardia: practical clinical points and comments on terminology.

Dextrocardia is defined by the authors as a right-sided heart with a base-apex axis directed rightward, resulting from a variation in cardiac development, and not used as a general term indicating any heart in the right chest. Dextrocardia occurs in approximately 0.01% of live births and can be discovered in various clinical settings and at various patient ages. The authors review their experience with dextrocardia, discuss useful clinical points that aid in evaluating complex anatomy, recount the history of dextrocardia terminology, and note the current inconsistent nomenclature.

Argatroban anticoagulation for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of heparin-induced thrombocytopenia.

Heparin-induced thrombocytopenia (HIT) is a life-threatening complication of heparin therapy. Anticoagulation in pediatric patients with HIT is challenging as there are no approved heparin substitutes. We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.

Modified De Vega mitral valve annuloplasty in an infant with endocarditis.

Surgical management of infant mitral valve regurgitation is challenging. There are no large surgical series of mitral valve repair in infants. Mitral valve repair is preferable to replacement. We report the modified De Vega adjustable annuloplasty with mitral valve repair in an infant with mitral valve regurgitation from endocarditis.

Prenatal detection of congenital heart disease in southern Nevada: the need for universal fetal cardiac evaluation.

OBJECTIVE: Congenital cardiac malformations are common developmental anomalies. In the United States, congenital heart disease is the number one cause of infant mortality from congenital malformations. Prenatal diagnosis of congenital heart defects aids treatment coordination. Our aim was to evaluate prenatal detection of serious congenital heart malformations in Clark County, Nevada. METHODS: We electronically searched our research department-maintained computer database for patients with serious congenital heart disease born in Clark County between May 2003 and April 2006. We excluded patients that did not have at least 1 local prenatal ultrasound examination. All pre-natal ultrasound studies were performed in obstetric offices, radiology imaging centers, or maternal-fetal medicine specialty practices. Fetal echocardiography was performed in maternal-fetal medicine specialists' offices under the supervision of a fetal cardiologist. Pediatric cardiologists performed all postnatal echocardiographic examinations. RESULTS: During the study period, we diagnosed serious congenital heart malformations in 161 patients among a total estimated 77,000 births (2/1000). Of the 161 patients, 58 (36%) had a prenatal diagnosis, and 103 (64%) had an exclusively postnatal diagnosis. CONCLUSIONS: Standard prenatal ultrasound fails to show congenital heart disease in most fetuses.

Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion.

OBJECTIVE: The purpose of this series was to assess the incidence, anatomic variants, and implications of an absent ductus venosus (ADV) in patients referred for fetal echocardiography. METHODS: We searched our fetal cardiology database for diagnoses of ADV from May 2003 to December 2006. RESULTS: During the study period, we performed 1328 fetal echocardiographic examinations in 990 fetuses. We found 6 cases of ADV (6/1000). Indications for fetal echocardiography were cardiomegaly, dilated umbilical or systemic veins, and extracardiac abnormalities. We identified 5 anatomic variants of ADV. In 2 patients, the umbilical vein connected to the systemic venous circulation by way of the portal sinus: via an abnormal venous channel from the portal sinus to the right atrium (case 1) and presumably via hepatic sinusoids to the hepatic veins (case 2). In the remaining 4 patients, the umbilical vein bypassed the portal sinus and the liver and connected to the systemic venous circulation via an abnormal venous channel: from the umbilical vein to the right atrium (case 3), from the umbilical vein to the inferior vena cava (cases 4 and 5), and from the umbilical vein to the right iliac vein (case 6). All patients survived; 2 required cardiovascular intervention. No intervention was required in 3 patients. CONCLUSIONS: An ADV should be ruled out in a fetus with unexplained cardiomegaly or dilatation of the umbilical vein, systemic veins, or portal sinus. To our knowledge, prenatal diagnosis of an ADV with an abnormal communication between the portal sinus and the right atrium has not been reported previously. The portosystemic communication persisted after birth and required device occlusion.