Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency.

We present a case report of a patient with conal septal hypoplasia (supracristal) ventricular septal defect (VSD) complicated by aortic insufficiency (AI) and subpulmonic stenosis from a fibrous membrane. The development of AI with VSD is a well-recognized problem. However, the association of VSD, AI, and significant right ventricular outflow tract obstruction (RVOTO) is less common. Mechanisms of RVOTO include prolapse of an aortic valve cusp across the VSD, as well as infundibular hypertrophy or muscle bundles. Technical echocardiographic issues can make the diagnosis of VSD, AI, and RVOTO challenging. The presence of a discrete fibrous subpulmonary membrane is uncommon in this setting.

Outcome after repair of tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression. METHODS: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years). RESULTS: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04). CONCLUSIONS: Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Prothrombotic effects of endothelin-2 (ET-2).

In this report we demonstrate that ET-2, in addition to its powerful vasoconstrictor properties and its role in hypertension, is capable of creating a hypercoagulable (prothrombotic) state. This study was facilitated by the utilization of a sensitive modified recalcification time (MRT) test that can measure coagulation at all points in the coagulation spectrum. The MRT was determined on aliquots (1 ml) of citrated blood which were added to saline (MRTS), to 10 micrograms of endotoxin (MRTE), and to 10 pg of ET-2 (MRT ET-2). The mean values of MRTS, MRTE, and MRT ET-2 were 4.3 +/- 0.8, 3.7 +/- 1.0, and 3.9 +/- 0.8 min, respectively. There was a statistically significant difference between MRTE vs. MRTS and MRT ET-2 vs. MRTS using the two-tailed t-test (p < 0.02 and p < 0.005, respectively).

Procoagulant property of platelet activating factor (PAF).

Platelet-activating factor (PAF) is known to activate platelets and thus promote clot formation in most mammalian species including humans. In this communication, the ability of PAF to hasten the overall coagulation process by measuring the recalcification time (RT) is described. Assessment of the RT in vitro indicated that the RT was significantly reduced by PAF (p < 0.001). This information adds a new dimension to the role of PAF in coagulation and also provides an exciting lead to the development of drugs for controlling the state of accelerated coagulation by pharmacologic means.