Complete agenesis of the right and left pulmonary arteries, with main pulmonary artery originating from the right ventricle, presenting as primary pulmonary hypertension of the newborn: case report.

A female infant who had been misdiagnosed as having primary pulmonary hypertension of the newborn was found to have agenesis of the branch pulmonary arteries with collateral-dependent pulmonary circulation. The intracardiac anatomy was completely normal. Unifocalization of branch pulmonary artery was done, followed by stenting of the ductus arteriosus.

Transvenous pacemaker insertion ipsilateral to chronic subclavian vein obstruction: an operative technique for children and adults.

Subclavian vein occlusion limits insertion of pacing electrodes in children and adults. The concern is greatest in children with a long-term need for pacing systems necessitating use of the contralateral vein and potential bilateral loss of access in the future. We describe an operative technique to provide ipsilateral access in chronic subclavian vein occlusion in five consecutive pediatric (n = 4, mean age 6.5 years) and adult (n = 1, age 70 with bilateral subclavian vein occlusion) patients in whom this condition was noted at the time of pacemaker or ICD implant. Occlusion was documented by venography. Pediatric cardiac diagnoses included complete heart block in all patients, tetralogy of Fallot in three, and L-transposition of the great vessels in one. Percutaneous brachiocephalic (innominate) or deep subclavian venous access was achieved by a supraclavicular approach using an 18-gauge Deseret angiocath, a Terumo Glidewire, and dilation to permit one or two 9-11 Fr sheaths. Electrode(s) were positioned in the heart and tunneled (pre- or retroclavicularly) to a pre- or retropectoral pocket. Pacemaker and ICD implants were successful in all without any complication of pneumothorax, arterial or nerve injury, or need for transfusion. Inadvertent arterial access did not occur as compared with prior infraclavicular attempts. One preclavicularly tunneled electrode dislodged with extreme exertion and was revised. Ipsilateral transvenous access for pacemaker or ICD is possible via a deep supraclavicular percutaneous approach when the subclavian venous obstruction is discovered at the time of implant. In children, it avoids the use of the contralateral vein that may be needed for future pacing systems in adulthood. This venous approach provides access large enough to allow even dual chamber pacing in children and can be accomplished safely.

Usefulness of triiodothyronine (T3) treatment after surgery for complex congenital heart disease in infants and children.

This is a study of the use of T3 infusion in the postoperative period in 6 pediatric patients who underwent complex cardiac surgical procedures under cardiopulmonary bypass. Normalization of serum T3 levels was reflected in a marked decrease in requirement of inotropic support, conversion to normal sinus rhythm, and progressively improving clinical course.

Aortic dissection: rupture into right ventricle and right pulmonary artery.

Rupture of an acute ascending aortic dissection into a surrounding cardiac chamber or pulmonary artery is an uncommon occurrence, and is often only diagnoses post mortem. Although fistulization (aortopulmonary and aorta-right atrial) after acute aortic dissection has been well documented in the literature, acute aortic dissection fistulizing into both the right ventricle and pulmonary artery has not. We report on a 75-year-old woman who presented with an acute ascending aortic dissection with both aortopulmonary and aorta-right ventricular fistulas who underwent repair and had long-term survival.

Intraaortic balloon rupture.

To identify patients at risk and assess management strategies, nine Datascope intraaortic balloon (IAB) ruptures in 8 patients were reviewed. This is a 2.4% incidence for this complication (382 insertions over 75 months). Time to initial rupture was 4.7 days, range 1.1 to 6.8 days. This was 1.3 days after the average intact device was removed. Women were three times more likely to sustain rupture than men (4 vs. 1.3%, P less than 0.05). Rupturing IABs were characterized by a greater magnitude of diastolic augmentation than intact IABs, 69.4 +/- 24.9 vs. 25.5 +/- 12.6 Torr (P = 0.01). A hypertensive treatment history was more prevalent in patients rupturing (83 vs. 41%) (P less than 0.05). Rupture was diagnosed by finding blood in the safety chamber in all patients. There was no gas embolization nor did any patient become infected. Ruptured devices showed abrasion wear in the most distal portion of the IAB, where the aorta is smallest. In vitro testing with CO2 showed no driving gas loss but withdrawal of the surrounding fluid into the IAB system. Ruptured IAB replacement in four patients was associated with three survivors. In four patients the device was not replaced and two patients survived. There were no complications as a consequence of IAB rupture (all values +/- SD).

Noninvasive pulse oximetry in children with cyanotic congenital heart disease.

Arterial oxygen saturation, determined noninvasively by pulse oximetry in 32 pediatric patients with cyanotic congenital heart disease (CHD), was compared with oxygen saturation measured by a cooximeter in simultaneously obtained arterial blood samples. The patients were studied in the cardiac catheterization laboratory, operating room, and ICU. Excellent correlation by linear regression (n = 108, r = .95) was observed between the two methods at oxygen saturations ranging from 35% to 95%. These observations show that in infants and children with cyanotic CHD, arterial oxygen saturations can be determined accurately and reliably by pulse oximetry at rest and during changing circulatory states.