Transcatheter closure of a giant communication between the right pulmonary artery and left atrium in a neonate.

Direct communication between the right pulmonary artery and the left atrium is an extremely rare congenital pathologic entity characterized by right to left shunt and a rare cause of cyanosis in newborn. The value of echocardiography is emphasized in making the diagnosis as well as never before reported successful treatment with the Amplatzer vascular plug in a neonate.

Repair of major congenital cardiac defects in low-birth-weight infants: is delay warranted?

OBJECTIVES: Few studies have described the survival of low-birth-weight infants weighing less than 1.5 kg at operation for a cardiac malformation. Our goal was to determine if body weight at surgery affects survival. METHODS: This was a retrospective cohort study using outcome data from the Pediatric Cardiac Care Consortium between 1982 and 2006. RESULTS: We reviewed the outcomes of 450 consecutive infants with a cardiac anomaly and a birth weight of less than 1.5 kg, and weight of less than 2.5 kg at surgery. Of these, 179 patients had undergone surgery with a weight of less than 1.5 kg and 271 patients weighed 1.5 to 2.5 kg at surgery. The 30-day survival rate was 83% for cohort 1 and 86% for cohort 2. For patients not requiring cardiopulmonary bypass, the 30-day survival rate was 86% for cohort 1 and 92% for cohort 2. For patients requiring cardiopulmonary bypass, the 30-day survival rate was 69% for cohort 1 and 73% for cohort 2. No notable improvement in the outcomes occurred over time. CONCLUSIONS: For low-birth-weight infants (weight < 1.5 kg) undergoing a major cardiac procedure, the survival of infants weighing less than 1.5 kg at surgery is comparable to that of infants who weighed 1.5 to 2.5 kg. We conclude that, in our series, weight was not an independent risk factor for mortality, and, therefore, operative delay because of patient weight might be unwarranted.

Mortality after total cavopulmonary connection in children with the down syndrome.

A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. Thirteen children without chromosomal aberrations were then selected as a control group, matched 1 to 1 for gender, age, weight, lesion, and type of Fontan procedure. The following variables were evaluated: pulmonary artery pressure and vascular resistance, weight, hemoglobin, degree of atrioventricular regurgitation, previous Glenn operation, fenestration, and length of stay in the hospital. In children with Down syndrome, mortality after the Glenn operation was 28%. Mortality after the Fontan operation was increased significantly (p = 0.001) in children with Down syndrome (35%) compared with those without Down syndrome (10%). Between patients with Down syndrome and controls, there were no significant differences in the perioperative parameters evaluated. Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery.

Initial Kaiser Permanente Southern California Experience Embracing the New Technology of Transcatheter Closure of Atrial Septal Defects.

As a result of individual physicians' initiative, transcatheter closure of secundum atrial septal defects-a new procedure-was made available to patients in the Kaiser Permanente (KP) Southern California Region soon after the US Food and Drug Administration (FDA) approved use of the AMPLATZER Septal Occluder. This ingenious device and the procedure for its implantation are described along with results of implantation in our initial 51 pediatric and adult patients. These results are compared with other published results. The clinical implications of using this new procedure are major: Many pediatric and adult patients with atrial septal defects can now benefit from nonoperative closure of these defects. On the basis of these observations, we attest to the commitment of Permanente physicians to incorporate technical advances into medical practice and to assess KP's experience using the new technology.

Chest pain during stenting of a native aortic coarctation: a case for acute intercostal muscle ischemia and rhabdomyolysis.

An adult male experienced severe chest pain during stenting of a native aortic coarctation. He also developed the postcoarctectomy syndrome with paradoxical hypertension and abdominal pain. Our hypothesis suggests that sudden interruption of large collateral blood flow caused acute chest wall muscle ischemia, rhabdomyolysis, and severe chest pain.