The evolution of the present approach to the newborn with hypoplastic left heart is outlined. Preoperatively, maintenance of ductal patency with prostaglandin E1 and balancing of systemic and pulmonary blood flow are essential. Operative details of the first-stage palliation and the definitive second-stage procedure are described. The more recent adoption of an intermediate-stage hemi-Fontan procedure is also described. Since January 1989, 151 patients have been treated using this three-stage approach, with 109 early survivors. Seventy-eight have undergone the hemi-Fontan operation with nine deaths (5 of whom came to this stage early nonelectively because of shunt failure or ventricular dysfunction). Twenty-seven of the 78 patients undergoing hemi-Fontan operation have subsequently undergone definitive Fontan procedures with no deaths.

BACKGROUND: Most of the ectopic beats initiating paroxysmal atrial fibrillation (PAF) originate from the pulmonary vein (PV). However, only limited data are available on PAF originating from the non-PV areas. METHODS AND RESULTS: Two hundred forty patients with a total of 358 ectopic foci initiating PAF were included. Sixty-eight (28%) patients had AF initiated by ectopic beats (73 foci, 20%) from the non-PV areas, including the left atrial posterior free wall (28, 38.3%), superior vena cava (27, 37.0%), crista terminalis (10, 3.7%), ligament of Marshall (6, 8.2%), coronary sinus ostium (1, 1.4%), and interatrial septum (1, 1.4%). Catheter ablation eliminated AF with acute success rates of 63%, 96%, 100%, 50%, 100%, and 0% in left atrial posterior free wall, superior vena cava, crista terminalis, ligament of Marshall, coronary sinus ostium, and interatrial septum, respectively. During a follow-up period of 22+/-11 months, 43 patients (63.2%) were free of antiarrhythmic drugs without AF recurrence. CONCLUSIONS: Ectopic beats initiating PAF can originate from the non-PV areas, and catheter ablation of the non-PV ectopy has a moderate efficacy in treatment of PAF.

Thirty-eight patients considered to be at increased risk for a Fontan repair underwent bidirectional cavopulmonary anastomosis. Twenty-one of the 38 had concurrent pulmonary artery reconstruction. Fontan risk factors included pulmonary artery distortion, elevated pulmonary artery resistance (greater than 2 Woods units) and/or pulmonary artery pressure (mean, greater than 18 mm Hg), atrioventricular valve regurgitation, systemic ventricular dysfunction, complex venous anatomy, and subaortic obstruction. There were no deaths, either early or late. Median arterial oxygen saturation increased from 79% to 84%(p less than 0.01). Median hospital stay was 8 days. No patient had pleural effusions after 7 days. Three patients had significant surgical complications. Five patients had inadequate relief of cyanosis; three of these had venous collaterals and two had severe ventricular dysfunction; the latter two patients subsequently had strokes. One of the patients with persistent cyanosis required a systemic-to-pulmonary artery shunt. We conclude that a bidirectional cavopulmonary shunt, with pulmonary artery reconstruction when indicated, provides adequate relief of cyanosis in most patients with single-ventricle lesions who are considered to be at increased risk for a Fontan repair. Relief of ventricular volume overload and pulmonary artery distortion may improve Fontan candidacy. Also, patients with persistent cyanosis after bidirectional cavopulmonary anastomosis should be catheterized for location and occlusion of venous collaterals. Further follow-up is necessary to determine the place of bidirectional cavopulmonary anastomosis in the management of patients at increased risk for a Fontan repair.

A specially devised pressure-sensitive valve forms the basis for a new peritoneo-venous shunt operation which delivers ascitic fluid continuously into the venous system. It is effective in ascites attributed to different causes. The procedure is simple and brings a long lasting relief with recovery in strength and nutrition and improved kidney function. Hepatorenal syndrome in ascites is reversed.

Fifty-four patients with renal cancer and vena cava tumour thrombus underwent radical nephrectomy and removal of the thrombus; the operative mortality rate was 9.3%(5 patients). The extent of the vena cava thrombus did not affect survival. Of 36 patients with no known pre-operative metastases and complete (29 patients) and incomplete (7 patients) removal of the vena cava tumour thrombus, the 5-year survival rate was 68 and 17%, respectively (P = 0.01). Thirteen patients (45%) who underwent complete removal of the vena cava tumour thrombus are alive and free of disease, with a mean follow-up of 51.2 months (range 4-144); three died without disease 110, 31 and 23 months after operation. The 2-year and 5-year survival rates of 18 patients with known pre-operative metastases was 37.5 and 12.5% respectively; 14 died between 1 and 27 months post-operatively (mean 11.6) of metastatic disease. Two of these 18 patients experienced long-term remission: one died of unrelated causes 151 months after operation; the other was lost to follow-up 219 months after operation, with no evidence of disease. Of 14 patients with positive regional nodes, the mean survival in those with metastases compared with those without metastases was 7.5 versus 15 months, respectively; only one patient survived at 14 months. Operative intervention in patients without metastatic disease (systemic or regional) and complete removal of the vena cava thrombus achieved a 5-year survival rate of 68%. Variables which significantly decreased survival and may be considered contraindications for operation were systemic metastasis, regional lymph node involvement and incomplete removal of the vena cava thrombus.

The bidirectional Glenn operation may be particularly useful as an intermediate procedure before Fontan correction in high-risk patients. From October 1989 through February 1992, 50 patients 1 to 60 months old (median 12) have undergone a bidirectional Glenn operation. Diagnoses included hypoplastic left heart syndrome in 21 patients, pulmonary atresia with intact ventricular septum in 10, tricuspid valve atresia in 9, other complex univentricular heart defects in 9, and Ebstein's anomaly in 1. Mean pulmonary vascular resistance was 2.2 +/- 0.2 Wood U (range 0.5 to 7.3) and mean pulmonary artery area Nakata index was 318 +/- mm2/m2 (range 80 to 821). Additional procedures were performed in 17 patients, including pulmonary artery reconstruction in 15 (29%) and bilateral caval anastomoses in 5 (10%). There were 4 hospital deaths (8%). Two deaths resulted from myocardial infarction in patients with pulmonary atresia with intact ventricular septum and sinusoids and 1 from severe pulmonary vascular disease in a patient with hypoplastic left heart syndrome. There was 1 late death from pneumonia. Actuarial survival is 92 +/- 4% at 1 month and beyond, with a mean follow-up of 13.4 +/- 1 months. Risk factor analysis showed that pulmonary vascular resistance > 3 Wood U and pulmonary artery distortion were associated with increased mortality. Twelve patients have undergone a Fontan procedure at a mean duration after bidirectional Glenn of 18 months with 1 death (8%). The bidirectional Glenn procedure provides excellent palliation in high-risk patients and appears useful as a staging procedure before Fontan correction.

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 and in a 26 day old infant with multiple cogenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals s short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well, the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.

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).

The first superior vena cava-pulmonary artery shunt (Glenn shunt) in our series was performed in February 1958. From then through September 1988, 91 patients have undergone this procedure for a wide variety of congenital defects. We here report follow-up data available on all patients. Ages ranged from 2 days to 46 years (mean 6.8). Diagnoses were as follows: tricuspid atresia, 27; single ventricle, 22; tetralogy of Fallot, 14; D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, 9; D-transposition, 5; Ebstein's anomaly, 4; pulmonary atresia + intact septum, 4; and others, 6. The hospital mortality rate was 7.7%(one death in the last 53 patients, 1.9%). Five deaths occurred in patients less than 6 months old. There were 20 late deaths (22%) with actuarial survival rates of 84% and 66% at 10 and 20 years, respectively. Pulmonary arteriovenous fistula formation was seen in 18 patients (19.7%), six of whom have undergone therapeutic embolization with improvement in saturation. The prevalence of pulmonary arteriovenous fistula increases with time after shunt. No long-term shunt thrombosis or stricture formation was seen. Fifty percent of shunts were still functioning at 20 years. Palliation was limited because of decrease in blood flow to the contralateral pulmonary artery, collaterals between the inferior and superior venae cavae, and pulmonary arteriovenous fistula formation. Improvement in saturation was obtained in eight otherwise inoperable patients by creation of a right axillary arteriovenous fistula up to 19 years after the Glenn shunt. Three patients had conversion of a Blalock-Taussig shunt to a Glenn shunt with improvement in congestive heart failure. Twenty-six patients have undergone a Fontan procedure with two deaths. Compared with the group having a Fontan procedure without a prior Glenn operation, there was no difference in early or late mortality. Thirty years after a Glenn shunt, the first patient in this series is working full time after having undergone a modified Fontan procedure in 1981. We conclude that the Glenn connection, usually with supplemental procedures to enhance oxygenation, has provided excellent physiologic palliation with low mortality up to 30 years with no late thrombosis or stricture formation. The incidence of pulmonary arteriovenous fistula increases with time and can be effectively treated with embolization. Physiologic repair after the Glenn shunt carries a low mortality. Although currently used infrequently, superior vena cava-pulmonary artery shunting remains a useful method of palliation in selected patients.(ABSTRACT TRUNCATED AT 400 WORDS)

BACKGROUND: Understanding the total cavopulmonary connection (TCPC) hemodynamics may lead to improved surgical procedures which result in a more efficient modified circulation. Reduced energy loss will translate to less work for the single ventricle and although univentricular physiology is complex, this improvement could contribute to improved postoperative outcomes. Therefore to conserve energy, one surgical goal is optimization of the TCPC geometry. In line with this goal, this study investigated whether addition of caval curvature or flaring at the connection conserves energy. METHODS: TCPC models were made varying the curvature of the caval inlet or by flaring the anastomosis. Steady flow pressure measurements were made to calculate the power loss attributed to each connection design over a range of pulmonary flow splits (70:30 to 30:70). Particle flow visualization was performed for each design and was qualitatively compared to the power losses. RESULTS: Results indicate that curving the cavae toward one pulmonary artery is advantageous only when the flow rate from that cavae matches the flow to the pulmonary artery. Under other pulmonary flow split conditions, the losses in the curved models are significant. In contrast, fully flaring the anastomosis reduced losses over the range of pulmonary flow splits. Power losses were 56% greater for the curving as compared to flaring. Fully flaring without caval offset reduced losses 45% when compared to previous models without flaring. If flaring on all sides was implemented with caval offset, power losses reduced 68% compared to the same nonflared model. CONCLUSIONS: The results indicate that preferentially curving the cavae is only optimal under specific pulmonary flow conditions and may not be efficient in all clinical cases. Flaring of the anastomosis has great potential to conserve energy and should be considered in future TCPC procedures.