Variation in the size and distensibility of the pulmonary arteries in d-transposition of the great arteries.

The relative size of the main pulmonary artery was determined from cineangiograms of 117 patients with d-transposition of the great arteries by calculating the ration between the diameters of the main pulmonary artery and aorta. The pulmonary artery was largest in patients with ventricular septal defect or patent ductus arteriosus, or both, because of increased pulmonary arterial pressure and flow. In patients with an intact ventricular septum or with left ventricular outflow tract obstruction, or both, the main pulmonary artery was approximately the size of the aorta. Two cases of d-transposition and gross dilatation of the main pulmonary artery and hypoplastic first branch pulmonary arteries are presented. In these cases the ratio between the diameters of the main pulmonary artery and aorta was greater than in any of the other 117 cases studied...

Analysis of survival in patients with pulmonic valve atresia and ventricular septal defect.

This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.

Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients.

Pulmonary vein wedge angiography was applied to 25 patients ranging in age from 4 months to 16 years. The indications for this technique include (1) determination of the presence or absence of central (intrapericardial or mediastinal) pulmonary arteries in patients with pulmonary atresia where standard anterograde injections of contrast medium do not demonstrate these, (2) determination of the patency and caliber of a nonvisualized pulmonary artery when there is distal "occlusion" by a systemic-to-pulmonary artery anastomosis, (3) determination of the patency and caliber of a nonvisualized pulmonary artery when previous pulmonary arterial banding has led to acquired pulmonary artery atresia, and (4) determination of the presence of a mediastinal pulmonary artery in patients with so-called isolated congenital unilateral absence of a pulmonary artery. Surgical correlates to the interpretation of pulmonary vein wedge angiograms are provided in 18 patients. The present data suggest that pulmonary vein wedge angiography is a safe technique that provides considerable information about (1) the caliber of the parenchymal pulmonary arteries, (2) their sizes at the hilum of the lung, and (3) the presence or absence of a mediastinal confluence of pulmonary arteries. This technique may also complement those anatomic findings derived from anterograde injection of contrast material in patients with pulmonary atresia of a congenital or acquired nature.

Bilateral ductus arteriosus (or remnant): an analysis of 27 patients.

Bilateral ductus arteriosus (DA) was clinically recognized in 27 patients studied angiographically from 1963 through May 1983. Distal bilateral DA origin of non-confluent pulmonary arteries was identified in 15 patients, ectopic or distal ductal origin of 1 pulmonary artery in 9 patients (5 without evidence of intracardiac disease) and isolation of the left subclavian artery in 3 (all 3 of whom had a right aortic arch). Other conditions reported to be associated with bilateral DA include interruption of the aortic arch with isolation of a subclavian artery, aortic atresia with interruption of the aortic arch in which bilateral DA supports the entire systemic circulation, bilateral DA complicating forms of congenitally malformed hearts other than those just stated, and, rarely, bilateral DA in isolation. Understanding the symmetric or paired nature of the primitive aortic arch system in the developing human heart facilitates recognition of the patterns of fourth and sixth arch anomalies seen with bilateral DA.

Retrobronchial vertical vein in totally anomalous pulmonary venous connection to the innominate vein and its specific occurrence in right isomerism.

Recently, a case of right isomerism with totally anomalous pulmonary venous connection (TAPVC) to the innominate vein through a vertical vein that ascended in the mediastinum posterior to the left-sided main bronchus was encountered. This retrobronchial vertical vein that has not been described previously in patients with TAPVC to the innominate vein prompted the performance of a retrospective analysis of 44 cases with TAPVC to the innominate vein in which angiography had been performed. The 44 cases consisted of 36 patients with situs solitus, 7 with right isomerism, and 1 with left isomerism. The spatial relation of the vertical vein to the adjacent pulmonary artery and main bronchus could be defined in 41 cases. The vertical vein was retrobronchial in 4 of 5 cases with right isomerism in which the location of the vertical vein could be defined with certainty. No case with situs solitus or left isomerism was associated with a retrobronchial vertical vein. In all 4 patients with a retrobronchial vertical vein, angiographic evidence of obstruction of pulmonary venous drainage was present, and was due to an extrinsic bronchial compression in 2 and to an intrinsic narrowing of the vertical vein in 2.

Double outlet right ventricle (S,D,L) with subaortic ventricular septal defect and pulmonary stenosis. Report of six cases.

The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was I-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle (S,D,L,). The ventricular septal defect to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis. The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic. Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.

Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum.

Twelve children with pulmonary atresia and intact ventricular septum underwent closure of the tricuspid valve as a part of a new surgical procedure. In two cases a concomitant Fontan operation was performed. In each patient the right ventricle was very small and right ventricular pressure was higher than systemic pressure. Ventricle-coronary connections provided flow of desaturated blood from the right ventricle into the coronary arteries in 11 of 12 cases. Five of the 12 children did not survive operation and postmortem examination of each revealed severe acute and chronic myocardial ischemic damage and high-grade obstruction or interruption of the proximal left anterior descending coronary artery. Preoperative angiography demonstrated occlusive changes in the coronary arteries, resulting in right ventricular dependent circulation, in all five children who died and in one child who survived operation. Seven children who survived operation are well 4 months to 3.5 years later. Two have undergone subsequent successful Fontan operation and two others are considered suitable candidates for this operation. Tricuspid valve closure is recommended for a carefully selected group of infants with pulmonary atresia and intact ventricular septum provided a right ventricular-dependent coronary circulation can be excluded on the basis of preoperative coronary cineangiography.

Congenital aortico-right atrial communications. The dilemma of differentiation from coronary-cameral fistula.

Four cases of an unusual form of aortico-right atrial communication are described. All patients were asymptomatic but had an atypical continuous murmur on examination. A distinctive appearance was noted on the angiograms, with a large tortuous tunnel noted superior to the left sinus of Valsalva, passing posterior to the aortic root before terminating near the right atrial-superior vena caval junction. This structure was readily identified by two-dimensional echocardiography. The defect was successfully closed surgically in three of four patients. The presence of normal major coronary arteries and absence of any small myocardial coronary branches from the tunnel argues against the structure being a coronary-cameral fistula and supports the diagnosis of aortico-right atrial tunnel.

Serous fluid leakage: a complication following the modified Blalock-Taussig shunt.

Polytetrafluoroethylene tubular grafts are useful in performing a systemic-to-pulmonary artery shunt (modified Blalock-Taussig). They allow a controlled-volume shunt, avoid kinking of the pulmonary artery, and preserve distal flow in the subclavian artery. Unfortunately, occasionally excessive serous fluid will drain through the interstices of the fabric. This results in excessive and prolonged chest tube drainage and/or localized seroma formation around the graft. This complication occurred in 26 children, an incidence of 18.8% of the 138 modified Blalock-Taussig operations. Repeat thoracotomy was required in four of these children--for evacuation of seroma mass in three and for relief of cardiac tamponade in one. Four other children required reinsertion of pleural or pericardial chest drains for excessive serous drainage persisting as long as 6 weeks after operation. Wrapping of the graft with silicone sheeting to facilitate subsequent takedown of the shunt led to seroma formation in five of nine children. We believe this practice should be abandoned. Excessive drainage of serous fluid through the interstices of PTFE grafts in almost 20% of our patients has compromised an otherwise satisfactory result of the modified Blalock-Taussig operation.

Subclavian arterioplasty. Repair of coarctation of the aorta in the first year of life.

Between June, 1977, and December, 1982, 106 infants underwent subclavian flap arterioplasty for repair of coarctation of the aorta in the first year of life. This technique was utilized because of our previous experience with the end-to-end technique in 181 infants, in whom there was a recurrence rate of 42% at 5 years. The recurrence rate in 78 of the 81 survivors who have been followed up for a mean of 18.8 months +/- 15.9 months (standard error) is 17.3% at 1 year and 11.1% at 2 and 3 years postoperatively. The presence of associated disease affects mortality: only one death in 34 patients with isolated coarctation; three deaths in 24 with coarctation plus ventricular septal defect; and 21 deaths in 45 infants with coarctation and complex lesions. Our present approach in the group with coarctation plus ventricular septal defect is to repair the coarctation without banding the pulmonary artery and then to close the defect if the infant remains in failure. Operative survival was 13 of 13 without pulmonary artery banding and 11 of 14 with pulmonary artery banding.

Results of the Fontan procedure for patients with univentricular heart.

One hundred twenty-four consecutive patients with univentricular heart undergoing the Fontan operation were reviewed. Patients with tricuspid atresia or biventricular heart with hypoplasia of one ventricle were excluded. Eighty-four patients had left ventricular morphology. Atrioventricular connection was double-inlet (n = 76), common (n = 29), absent left atrioventricular connection (n = 14), and absent right atrioventricular connection (n = 5). Actuarial survival was 77% (70% confidence limits, 73% to 81%) at 1 year, 66% (70% confidence limits, 60% to 72%) at 5 years, and 49% (70% confidence limits, 36% to 61%) at 10 years, indicating a continuing risk for premature death. Multivariate analysis identified preoperative ventricular function and hypertrophy as risk factors for survival. High postrepair right atrial pressure (greater than 15 mm Hg) emerged as a strong intraoperative predictor of survival. Logistic regression analysis of these factors predicts high probability of death for certain subgroups of patients after the Fontan operation. Forty-four percent (n = 53) of these original 124 patients are alive and in New York Heart Association class I at follow-up. Thirty-eight percent (n = 33) of survivors have worse ventricular function than preoperative. Long-term survival is disappointing. Certain identifiable subgroups of patients with univentricular heart have unacceptable risks for the Fontan operation and should have alternate management. High postrepair right atrial pressure is an ominous sign, and if it persists the Fontan should be fenestrated or taken down.

The investigation of abnormal pulmonary arteries in congenital heart disease.

Thorough investigation of the state of the pulmonary vascular bed is an important part of the preoperative assessment of patients with congenital heart disease. This article describes the imaging techniques applicable to anomalies and acquired changes of the pulmonary arteries, with emphasis on recent developments. The morphology and imaging of specific cardiovascular anomalies that involve the pulmonary arteries are described and illustrated.

A case report of double-orifice tricuspid valve.

A heart specimen with double-orifice tricuspid valve is described. The tricuspid valve was divided into the anterior and posterior orifices by a bridge of leaflet tissue. The valve was stenotic because of the arcade deformity of the anterior papillary muscle to which the bridging leaflet tissue had short chordal insertions. The anterior orifice was regurgitant as a result of a deficient septal leaflet. Clinical correlation was obtained by magnetic resonance imaging of the specimen. The functional significance of the malformed valve was assessed by three-dimensional reconstruction of the two-dimensional magnetic resonance images.

Surgical experience with left ventricular outflow tract obstruction in patients with complete transposition of the great arteries and essentially intact ventricular septum undergoing the Mustard operation.

During a 24-year period (1963-1987), 46 infants and children with complete transposition of the great arteries (CTGA) and an essentially intact ventricular septum (IVS) have undergone surgical relief of left ventricular outflow tract obstruction (LVOTO) concurrently with the Mustard operation (MO). The obstruction was valvar in 2 patients, subvalvar in 43 and combined valvar and subvalvar in 1; the ventricular septum was intact in 42, and 4 patients had a small VSD at the time of repair. Neonatal and pre-Mustard cardiac catheterization (CC) data were analyzed to obtain LV to right ventricular peak systolic pressure ratio (LV/RVPSP) and LVOT peak systolic gradient (PSG). The mean neonatal LV/RVPSP (n = 31) was 0.79 +/- 0.04; pre-Mustard LV/RVPSP (n = 44) was 0.75 +/- 0.05; pre-Mustard LVOT PSG (n = 34) was 46.5 +/- 3.5 mmHg. Neonatal (n = 29) and pre-Mustard (n = 30) cardiac angiograms were retrospectively reviewed to analyse morphologic substrates of LVOTO. In the neonatal period, LVOTO was present in 7 patients (anatomic, n = 4; dynamic, n = 2; combined, n = 1). Immediately prior to the MO, LVOTO was present in 29 (anatomic, n = 20; dynamic n = 2; combined, n = 7). Techniques to correct LVOTO at the initial MO included pulmonary valvotomy (n = 3), ventriculomyectomy (n = 41), resection of an windsock aneurysm (n = 1), LV to pulmonary artery valved conduit (VC) (n = 1). Two patients underwent VC early postoperatively (PO) for severe residual LVOTO (both died).(ABSTRACT TRUNCATED AT 250 WORDS)