Danielson, Gordon K.

Six years have passed since the first successful surgical correction of truncus arteriosus. A review of our experience enables some conclusions regarding the operation. Patients with mild or moderate truncal valve incompetence do not need truncal valve replacement. Patients with severe truncal valve incompetence require valve replacement, which is associated with a significantly increased surgical mortality. The surgical mortality is not increased in hemodynamically favorable patients who have only one pulmonary artery. However, these patients are especially likely to have early development of severe pulmonary vascular disease. The surgical mortality for the patient with uncomplicated disease and two pulmonary arteries, with pulmonary resistance of less than 8.0 units m 2 , is 10%. In patients with pulmonary resistance between 8.0 and 12 units m 2 , the mortality is approximately three times greater. Patients with pulmonary resistances greater than 12.0 units m 2 are probably inoperable. Different hemodynamic criteria must be applied in assessing the operability of patients with a single pulmonary artery. A systemic arterial oxygen saturation less than 85% in a patient with two pulmonary arteries and without pulmonary artery stenosis or a pulmonary artery band usually indicates inoperability. Elective operation usually is deferred until a patient is four years old, but if the patient's clinical condition warrants, the procedure can be done at any time after the age of one year, with a good chance of success. Follow-up on most operated patients has been encouraging.

From 1957 through 1971, 18 patients whose ages ranged from 10 months to 18 years underwent surgical correction of tetralogy of Fallot with absent pulmonary valve. All patients had associated pulmonary arterial aneurysms; in two the left pulmonary artery also was absent. Right middle lobectomy had been performed previously on one patient for lobar emphysema; seven other patients had recurrent pulmonary infections. The ventricular septal defect was closed with a patch in 12 patients and by direct suture in 6. Concomitant infundibular resection was done in all. In four the narrow pulmonary valve annulus was enlarged with an outflow patch, and multiple radial incisions were made in the valve annulus in seven others. The pulmonary arterial aneurysms were not resected in any patients. There were three hopsital deaths (17%) and two late deaths. Complete heart block developed intraoperatively in three patients, all operated on prior to 1963, contributing to the early death of one patient and to the late deaths of the other two. In a follow-up of all 13 survivors at 8 months to 15 years (median 6 years), results were excellent and equal to those of patients who have undergone repair of tetralogy of Fallot with pulmonary valve.

Fifty patients underwent correction of one or two valvular lesions with concomitant insertion of one to three aortocoronary vein bypass grafts. Hospital mortality was 14% and late mortality was 10%. Factors influencing operative mortality were: (1) mitral valve replacement after myocardial infarction; (2) complete obstruction of a coronary artery preoperatively; (3) high left ventricular end-diastolic pressure at rest preoperatively in patients with mitral valve disease; and (4) use of a single aortocoronary graft in the presence of multiple-vessel disease. For patients with significant associated valvular and coronary artery disease, combined correction yields results that appear sufficiently encouraging, judged by mortality and follow-up, to warrant its continued use in selected cases.

The results of complete repair of double-outlet right ventricle with pulmonic stenosis in 22 Mayo Clinic patients were analyzed. The overall mortality rate was 32%. Since 1960, the mortality rate has been reduced to 16%. Anomalies of the distribution of the right coronary artery, a second ventricular septal defect of the atrioventricular canal type, and significant residual pulmonic stenosis were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Complete repair is best carried out after the patient is 4 years old. A systemic-to-pulmonary arterial shunt is the procedure of choice for those who are younger than this. In the occasional patient with anomalous distribution of the right coronary artery which precludes adequate relief of the pulmonic stenosis, use of an aortic homograft from the right ventricle to the pulmonary artery should be considered.

The results of complete repair of double-outlet right ventricle without pulmonic stenosis in 18 Mayo Clinic patients are analyzed; the over-all operative mortality rate was 22%. Pulmonary vascular obstructive disease and a second ventricular septal defect of the atrioventricular canal type were associated with a higher mortality rate. The difficulties in the diagnosis of this uncommon condition are emphasized. Patients with double-outlet right ventricle without pulmonic stenosis should have complete repair early in life before the onset of severe pulmonary vascular obstructive disease-the only specific contraindication to operation. Pulmonary artery banding may be preferable as a first-stage palliative procedure in infants less than six months old who do not respond to medical therapy.

Four cases of ventricular septal defect and ventricular aneurysm after myocardial infarction are reported. The clinical findings and surgical results in these four cases and in eight previously reported patients with this combination of complications are summarized. Resection of the ventricular aneurysm and closure of the ventricular septal defect appear indicated, both on theoretical grounds and in view of the excellent results observed in six patients who underwent combined repair. In one of our patients, closure of the ventricular septal defect and resection of the ventricular aneurysm were possible with only a single suture line, as these defects were anatomically contiguous.

Background —The clinical spectrum of constrictive pericarditis (CP) has been affected by a change in incidence of etiological factors. We sought to determine the impact of these changes on the outcome of pericardiectomy. Methods and Results —The contemporary spectrum of CP in 135 patients (76% male) evaluated at the Mayo Clinic from 1985 to 1995 was compared with that of a historic cohort. Notable trends were an increasing frequency of CP due to cardiac surgery and mediastinal radiation and presentation in older patients (median age, 61 versus 45 years). Perioperative mortality decreased (6% versus 14%, P =0.011), but late survival was inferior to that of an age- and sex-matched US population (57±8% at 10 years). The long-term outcome was predicted independently by 3 variables in stepwise logistic regression analyses: (1) age, (2) NYHA class, and most powerfully, (3) a postradiation cause. Of 90 late survivors in whom functional class could be determined, functional status had improved markedly (2.6±0.7 at baseline versus 1.5±0.8 at latest follow-up [ P <0.0001]), with 83% being free of clinical symptoms. Conclusions —The evolving profile of CP, with increasingly older patients and those with radiation-induced disease in the past decade, significantly affects postoperative prognosis. Long-term results of pericardiectomy are disappointing for some patient groups, especially those with radiation-induced CP. By contrast, surgery alleviates or improves symptoms in the majority of late survivors.

Background Conventional cardiac catheterization criteria for the diagnosis of constrictive pericarditis (CP) rely on equalization of intracardiac pressures and have many recognized limitations. Recently, Doppler echocardiographic methods have been used to examine dynamic respiratory changes of increased ventricular interdependence and dissociation of intrathoracic and intracardiac pressures for the diagnosis of CP. These pathophysiological features may be best delineated by cardiac catheterization. Therefore, we studied the accuracy of these dynamic respiratory changes in left ventricular and right ventricular pressure for the diagnosis of CP at cardiac catheterization. Methods and Results High-fidelity manometric catheters and respirometry were used to study 36 patients: 15 patients with surgically proven CP (group 1) and 21 patients with other causes of heart failure (group 2). Conventional cardiac catheterization variables used to establish the diagnosis of CP lacked sensitivity and specificity and failed to distinguish between these groups. However, the finding of discordance between right ventricular and left ventricular pressures during inspiration, a sign of increased ventricular interdependence, accurately distinguished patients in group 1 from those in group 2 ( P <.05). Conclusions Examination of dynamic respiratory changes indicating increased ventricular interdependence may be helpful in the diagnosis of CP in the cardiac catheterization laboratory.

BACKGROUND The purpose of this study was to estimate survival and quality of outcome and assess factors associated with outcome for patients out 5 to 15 years from their Fontan operation. METHODS AND RESULTS We studied 352 patients who had the Fontan operation prior to 1985. The overall 1-, 5-, and 10-year survival was 77%, 70%, and 60%, respectively. The following factors were significantly associated with lower survival: univentricular heart or complex congenital anomalies other than tricuspid atresia, early calendar year of operation, heterotaxia syndromes, early age at operation, increased pulmonary artery pressure, atrioventricular valve dysfunction, and higher (worse) New York Heart Association class. Reoperations were necessary for 103 of the 352 patients. At least 20% of the survivors have or have had cardiac arrhythmias requiring antiarrhythmic medication or mechanical pacemaker insertion. Between 7% and 10% of the patients have had or had protein-losing enteropathy/hypoproteinemia. At 5 years postoperatively, 122 patients (34.7%) were alive with a better New York Heart Association functional classification than preoperatively. Fifty-eight patients (16.5%) were alive and in the same functional classification, but 126 (35.8%) died within the first 5 years or were in a worse functional classification. Thirty-nine patients were doing excellently and 29 patients poorly 5 years after the operation. Of the surviving patients, 43% can do as much exercise as their peers, whereas 3% are incapable of exercise. CONCLUSIONS To assure good functional long-term outcome in addition to survival, clinicians must exclude from selection for Fontan operation patients known to be at high risk for death or poor outcome.

In a group of 191 consecutive patients who underwent operation at the Mayo Clinic for isolated pulmonary valve stenosis between 1956 and 1967, late results were excellent. In patients surviving operation before the age of 21 years, long-term survival was similar to that in an age- and sex-matched control population. In the other patients, late survival and functional status were good, but survival was poorer than in the control population. This late attrition, most likely due to the sequelae of long-standing right ventricular hypertrophy, suggests the need for continued surveillance. Late sudden death occurred in only three patients, between 10 and 21 years postoperatively. The finding of normal life expectancy for such patients who survive operation before age 21 is important for actuarial purposes and for the patients' career and employment plans.