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Biomedical subjects

J D Waldman

Publications and source records attributed to J D Waldman.

At least 19 recordsLinked to original sources

The Damus-Fontan procedure.

The Damus-Kaye-Stansel operation is a useful technique for the treatment of complex cyanotic congenital heart disease when there is obstruction between the systemic ventricle and the aorta. Modifications of the technique include transection of the aorta and the pulmonary artery, anastomosis of the contiguous aortic and pulmonary walls, and connection of the distal aorta to the perimeter of the new bivalved proximal great artery. In addition, the bidirectional cavopulmonary shunt technique can be used with or without the Fontan procedure. Six patients underwent a Damus-Fontan operation, and all survived. Two patients underwent the Damus-cavopulmonary shunt (hemi-Fontan) procedure, and 1 survived. The postoperative status of the 7 survivors is good to excellent. Follow-up ranges from 2 months to 7 1/2 years.

Anastomosis, Surgical

The bidirectional cavopulmonary shunt.

The bidirectional cavopulmonary shunt improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance. Since 1983, 17 patients have undergone a cavopulmonary shunt procedure (five primary operations, 12 secondary operations). Diagnoses were single ventricle complex (n = 4), hypoplastic right heart syndrome (n = 10), and hypoplastic left ventricle (n = 3). Age at primary operation ranged from 3 1/2 to 30 months (median 6 months). Weight ranged from 3.5 to 9.7 kg. Age at secondary operation ranged from 10 months to 14 years (median 15 months). Seven cavopulmonary shunt operations were performed without cardiopulmonary bypass (six via thoracotomy and one via sternotomy) and 10, with cardiopulmonary bypass. All patients in the bypass group had additional procedures: takedown of modified Blalock-Taussig shunt, seven patients; revision of right ventricular outflow tract, four patients; reconstruction of pulmonary arteries, four patients; tricuspid valvuloplasty, one patient; and Damus procedure, one patient. There was one (1/17) operative death (Damus procedure). One patient required early revision. Follow-up ranges from 1 to 53 months (median 23 months). Twelve of 16 had a good to excellent late result, with a rise in mean arterial oxygen saturation from 69% to 83%. Three patients died late (4 to 53 months) (pulmonary vascular disease, pulmonary arteriovenous malformations, and pneumonia, one patient each). There was one late failure (converted to Glenn shunt). The cavopulmonary shunt is an excellent palliative procedure when right atrium-pulmonary artery connection (modified Fontan) must be deferred because of age, weight, or anatomic considerations. Five patients have undergone right atrium-pulmonary artery connection later. In addition, at the time of the modified Fontan operation, the cavopulmonary shunt approach may optimize the anatomic connection (eight additional patients).

Adolescent

Therapeutic cardiac catheterization in children.

Cardiac catheterization, once the mainstay of diagnosis in children with congenital heart disease, has become a therapeutic modality for many conditions. Balloon dilatation can now open stenotic valves and vessels, coils and umbrellas can now close unwanted communications, and emboli can be withdrawn without surgical intervention.

Cardiac Catheterization

Late reoperation for systemic atrioventricular valve regurgitation after repair of congenital heart defects.

Since 1979, 17 infants and children have undergone reoperation for systemic atrioventricular (AV) valve regurgitation 6 weeks to 7 years after repair of congenital heart defects. Prior operations were repair of incomplete or complete AV canal (14 patients), Mustard repair of complex transposition of the great arteries including ventricular septal defect closure (2 patients), or first-stage operation for hypoplastic left heart (1 patient). Age ranged from 6 months to 11 years. In 12 of the 17 patients (10, AV canal; 1, transposition; 1, hypoplastic left heart), valve reconstruction was possible. Operative techniques included a combination of septal cleft approximation, leaflet resection, commissural annuloplasty, or ring annuloplasty. There were no operative deaths, and there were no reoperations in the repair group. The condition of these patients has improved. Follow-up ranges from 1 month to 9 years (mean follow-up, 4.1 years). Five of the 17 patients (4, AV canal; 1, transposition) underwent valve replacement. There were no operative deaths. Follow-up ranges from 3 to 8 years. Three patients later underwent re-replacement of the prosthetic valve; there was 1 late death. The condition of all 4 survivors is improved. Substantial AV valve regurgitation can occur months or years after repair of congenital heart defects. A combination of reconstructive techniques may be useful in preserving native valve function and avoiding systemic AV valve replacement.

Child

The cryopreserved homograft valve in the pulmonary position: early results and technical considerations.

Since September, 1985, 20 patients have undergone implantation of a homograft valve in the pulmonary position (16 pulmonary, 4 aortic). There were 11 primary operations and 9 reoperations. In 7 of 11 primary operations the homograft valve was utilized as a composite conduit with a short Dacron extension. In four of five reoperations for a failed porcine valved conduit, a composite homograft conduit was used. Four patients underwent implantation of a free homograft in a previously repaired right ventricular outflow tract (RVOT). Age ranged from 15 days to 22 years. There was one operative death (5%), a seven-week-old infant with truncus arteriosus. Long-term follow-up ranges from 1 to 30 months. Clinical performance has been satisfactory in 18 of 19 patients. One patient undergoing free implantation of a pulmonary valve in the RVOT required replacement at 18 months with a porcine valve. In this patient, pulmonary insufficiency was caused by distortion of the annulus secondary to dilatation and pulmonary hypertension. Nine of 18 survivors do not require medication. Eleven of 18 have trivial to mild pulmonary insufficiency murmurs without symptomatology. The homograft valve is extremely useful in reconstruction of the right heart, however, early insufficiency murmurs have been noted. Distortion of the valve annulus may contribute to the early onset of a benign insufficiency murmur. Residual distal obstruction or pulmonary hypertension may be a contraindication to the use of a free homograft in the orthotopic position.

Adolescent

Balloon dilatation of stenotic right ventricle-to-pulmonary artery conduits.

In seven children with obstructed right ventricle-to-pulmonary artery conduits, balloon dilatation (BD) was performed 10-58 months after insertion of the conduit. Average valve gradient was reduced from 69 to 32 mmHg. Obstruction at the conduit-branch pulmonary artery connection became apparent after dilatation of the valve; these distal stenoses also were dilated. It is concluded that the useful lifetime of a right ventricle (RV) to pulmonary artery (PA) conduit may be extended by BD of an obstructed biological valve and/or BD of a stenotic conduit-pulmonary artery anastomosis.

California

Experience with Damus procedure.

Sixteen patients with complex cyanotic congenital heart disease underwent an operation involving the use of the aorta and the main pulmonary artery as the egress for systemic arterial blood flow (the Damus-Kaye-Stansell operation, modified as discussed). The indication for this approach was either preoperative aortic outlet obstruction or likelihood that the repair would produce such obstruction. A modification of the original technique is introduced that involves transection of both great arteries, anastomosis of the contiguous aortic and pulmonic walls, and connection of the distal aorta to the perimeter of the new (bivalved) great artery. Eleven of the 16 patients survived operation: six of six who underwent a concurrent Fontan operation, four of six who underwent a concurrent right-ventricle-to-pulmonary-artery-conduit procedure, and one of four who underwent a concurrent systemic-pulmonary shunt procedure. Postoperative status in the 11 survivors is good: one patient has a gradient from the left ventricle to the aorta, 10 of 11 have little or no aortic regurgitation, and the remaining patients have a normal physiological status. We conclude that the Damus procedure is a useful addition to the surgical armamentarium for treatment of certain types of cyanotic congenital heart disease.

Angiography

The conus with no great artery.

In three patients with complex cyanotic congenital heart disease, the conus was found to have no arterial outlet. The semilunar valves were at similar, caudad levels, and there was malposition of the great arteries. The aorta was related to the body--not the outflow portion--of the right ventricle in two patients with transposition and to the body of the left ventricle in one child with single ventricle. These cases confirm the independent embryological segregation of the conus and the truncus. Failure to recognize this condition can lead to erroneous relationship diagnoses and may adversely influence surgical decisions.

Arteries

Balloon dilatation of porcine bioprosthetic valves in the pulmonary position.

Balloon dilatation (BD) of bioprosthetic valves was investigated in vivo and in vitro. Four children with stenotic bioprosthetic porcine valves in the pulmonary position underwent BD of the valve 10 to 24 months after its insertion. Average valve gradient was reduced from 47.5 to 27 mm Hg. Obstruction at the conduit-branch pulmonary artery connection became apparent after dilatation of the valve. These distal stenoses were also dilated. BD technique was tested in vitro with the use of nonstenotic valves in fresh conduits. No damage to the valve or to the conduit was found when oversized balloons were used in a standard fashion or intentionally inflated until rupture. It is concluded that conduit replacement may be deferred by balloon dilatation of obstructed biological valves and/or a stenotic conduit-pulmonary artery anastomosis.

Angioplasty, Balloon

Anastomosis of systemic veins to pulmonary arteries for physiologic repair of asplenia syndrome.

In a patient with single ventricle, mitral atresia, common atrium, pulmonary stenosis, azygos continuation of the inferior vena cava and bilateral systemic artery--pulmonary artery anastomoses, the systemic veins were connected directly to the pulmonary arteries to achieve physiologic repair; the child was discharged five days after surgery and is asymptomatic three years postoperatively. This case illustrates the usefulness of azygos continuation of the inferior vena cava and emphasizes the need for low pulmonary artery pressure when a cavopulmonary anastomosis is done.

Angiography

An autosomal dominant syndrome of characteristic facial appearance, preauricular pits, fifth finger clinodactyly, and tetralogy of Fallot.

This report describes six relatives with a syndrome of mild prenatal-onset growth deficiency, an altered craniofacial appearance, preauricular pits, and clinodactyly of the fifth finger; three had tetralogy of Fallot. The clinical impact of this condition appears to be related to the severity of the cardiac defect. Autosomal dominant inheritance is implied by the occurrence of the disorder in three successive generations with documented male-to-male transmission. Recognition of this syndrome is important in counseling families regarding recurrence risk for tetralogy of Fallot.

Abnormalities, Multiple

Early extubation after surgical repair of congenital heart disease.

Of 220 pediatric patients recovering from surgical repair of congenital heart disease, 147 (67%) met our criteria for early extubation and were extubated either in the operating room or within 6 h after admission to the surgical ICU. The duration of postoperative mechanical ventilation correlated with the duration of cardiopulmonary bypass during surgical repair. In patients undergoing repair of ventricular septal defect, preoperative measures of pulmonary vascular status (PPA/PAO and RP/RS) also correlated with the duration of mechanical ventilation.

Adolescent

"Zero reference" during cardiac catheterization.

Hemodynamic pressure measurements obtained during cardiac catheterization studies are calibrated to an arbitrary level where the pressure is considered zero. Despite the fundamental nature of the zero reference level, there is no standardized method for determination of "zero reference." A horizontal metal rod has been constructed to be level with the pressure transducer domes. With lateral radiographic viewing, the metal rod is adjusted to be level with the junction of the inferior vena cava and the right atrium. The technique is rapid, reliable, without risk and allows accurate comparison of hemodynamic data between patients and in the same patient longitudinally.

Blood Pressure

Angulation of the inferior vena cava-right atrial junction in children with repaired omphalocele.

Three children with repaired omphalocele underwent diagnostic cardiac catheterization. In addition to intracardiac shunts, each was found to have marked angulation at the junction of the inferior vena cava and the right atrium. After review of our autopsy material following omphalocele repair, it is suggested that the inferior vena cava - right atrial angulation is due to gradual abnormal fixation of this junction either in utero or related to the surgical repair. A change in current surgical therapy is NOT recommended. Angulation of the inferior vena cava - right atrial junction is potentially important because a) it may interfere with venous cardiac catheterization from "below", b) it poses a danger to inferior vena caval cannulation for cardio-pulmonary by-pass, and c) it may become obstructive with time producing portal hypertension.

Cardiac Catheterization

Antegrade left heart catheterization.

Retograde arterial catheterization has been the standard approach to hemodynamic and angiographic evaluation of the left ventricle and systemic arterial system. An alternative approach--antegrade left heart catheterization--is reported that circumvents arterial puncture. During cardiac catheterization from the femoral vein in 18 children with normally related great arteries who had interatrial communications, a balloon-tipped catheter was looped in a "clockwise" fashion through the left atrium and left ventricle and into the ascending aorta. In 17 children the maneuver was rapidly accomplished without complications; it proved possible to perform in an infant with a hypoplastic left ventricular cavity, and was employed to perform a renal arteriogram. The major advantage of antegrade left heart catheterization is avoidance of complications related to arterial puncture. The antegrade approach also avoids right arm cut-down in children with coarctation of the aorta.

Aorta