Results of the modified Fontan operation for congenital heart lesions in patients without preoperative sinus rhythm

Preoperative sinus rhythm has been a criterion for the Fontan operation. However, of 297 patients who underwent the Fontan operation between October 1973 and February 1984, 12 (4%) did not have sinus rhythm. The age at operation ranged from 4 to 34 years (median 15). Nine patients had a univentricular heart, two had tricuspid atresia and one had a complex form of transposition. In all 12 patients, 3 to 8 of the 10 proposed criteria for operability were not met. An atrioventricular (AV) conduction abnormality was present in seven patients, six with complete AV block and one with AV dissociation. The patient with complex transposition had complete AV block and atrial fibrillation. Postoperatively, all seven patients continued to have an AV conduction abnormality, and those with complete AV block had a permanent pacemaker implanted. Six of the 12 study patients had atrial flutter or fibrillation refractory to antiarrhythmic medications. Postoperatively, four of the six patients had sinus rhythm. Two of the six patients had complete AV block (including the patient with complex transposition) and both had a permanent pacemaker implanted. Three of the 12 patients died (mortality rate 25%). The nine survivors were followed up for 6 to 55 months; no late deaths occurred. All had marked clinical improvement. This study demonstrates that 1) complete AV block is not a contraindication to the Fontan operation, 2) some patients may not require AV synchrony postoperatively for survival, and 3) postoperative atrial flutter or fibrillation may cease or be easier to control after the Fontan operation.     

Idiopathic hypertrophic subaortic stenosis in the young

Thirty-six young patients with idiopathic hypertrophic subaortic stenosis were studied. Twenty-seven patients were male and 9 female, and their mean age was 11.3 years (range 5 months to 20 years). Twenty-three patients (64 percent) had symptoms, the most common being dyspnea, angina and syncope. Diagnostic difficulties were encountered frequently in younger patients, especially those with right heart involvement, and in asymptomatic patients with murmurs suggestive of other cardiac defects. Patients were classified retrospectively into three groups on the basis of management. The first group consisted of 16 patients who were operated on; 4 of these patients died, 1 operatively and 3 suddenly late postoperatively (at 1.6, 2 and 10 years). The 12 long-term survivors (average follow-up period 6.2 years) have had good relief of symptoms. The second group comprised seven patients treated with propranolol; none of these died. The 13 patients in the third group received no therapy; 7 of these patients died, 6 suddenly and 1 from congestive cardiac failure. Idiopathic hypertrophic subaortic stenosis is a serious disorder that may present at any age and that may be difficult to diagnose. All patients with this disorder should be treated with propranolol; surgical intervention, although it does not totally abolish the risk of sudden death, appears to offer symptomatic improvement in most cases over a long-term follow-up period.     

Right and left ventricular function after the Mustard procedure in transposition of the great arteries.

Right and left ventricular function was assessed at cardiac catheterization in 33 asymptomatic patients 0.5 to 11 years (mean 4.6) after the Mustard operation for complete transposition of the great arteries. Ages at operation had ranged from 0.5 to 16 years (mean 4.2 years). Right ventricular function was assessed using videodensitometric determination of ejection fraction and ventricular volume data. Ventricular volumes were obtained by computerized video analysis utilizing Simpson's rule. The right ventricular ejection fraction was 37 +/- 11 percent (standard deviation), as assessed with videodensitometry and 42 +/- 10 percent as assessed with ventricular volume--both values less than normal (P less than 0.001). Right ventricular end-diastolic volume was significantly greater than normal (P less than 0.001) and averaged 202 +/- 70 percent, but left ventricular end-diastolic volume averaged only 125 +/- 53 percent. These observations after the Mustard operation indicate that right ventricular function is seriously decreased with relatively preserved left ventricular function. They support efforts for surgical correction utilizing the left ventricle as the systemic ventricle.     

Cardiac receptors: normal and disturbed function.

Widely distributed throughout the heart is a network of fibers connected to the medullary cardiovascular centers by nonmedullated vagal afferent fibers. When the traffic in these fibers is interrupted by vagal cooling, and the input from the arterial baroreceptors is prevented, the arterial blood pressure increases. Thus, these receptors act to inhibit tonically the vasomotor center. The receptors in the atria alter their rate of discharge with changes in atrial transmural pressure and contractility and are most active during end-inspiration and early expiration when the transmural pressure is maximal. The receptors in the ventricles respond to changes in ventricular end-diastolic pressure (preload), to the pressure generated during systole (afterload) and to changes in ventricular contractility. The cardiac mechanoreceptors have an equal or greater effect on the renal bed than the arterial mechanoreceptors and this effect is enhanced by hypercapnia. In animals, the cardiac mechanoreceptors have less control of the muscle vessels than the arterial mechanoreceptors, but the reverse is true in man. Both the cardiac and arterial mechanoreceptors can modulate the output of renin from the kidney, but the cardiac mechanoreceptors are more sensitive to small changes in blood volume. During coronary occlusion, in association with the bulging of the ischemic myocardium, the rate of discharge of these cardiac receptors is greatly increased.     

Idiopathic hypertrophic subaortic stenosis: Long-term surgical follow-up

The results of surgery in 43 patients with idiopathic hypertrophic subaortic stenosis are reported. Twenty-eight patients were male and 15 female; their mean age was 34 years. Dyspnea, angina pectoris and dizziness or syncope were the most common symptoms, in decreasing frequency. Before the operation, 3 patients were in New York Heart Association functional class I, 9 in class II and 31 in classes III and IV. The mean resting peak ventricle-arterial systolic pressure gradient was 77 mm Hg (39 patients), and mean left ventricular end-diastolic pressure was 22 mm Hg (34 patients). The operative mortality rate was 16 percent (7 of 43 patients) for the entire group, but only 8 percent for patients who underwent isolated left ventricular outflow tract myectomy (3 of 36 patients). There were four late sudden deaths. One patient has been lost to follow-up. The remaining 31 patients have been followed up for a mean of 84 months (range 12 to 160 months); 14 patients are in functional class I, 14 in class II and 3 in classes III and IV. Of the symptomatic patients, six are taking propranolol and eight are taking digitalis or diuretic agents, or both. Complications included complete heart block in 5 patients, complete left bundle branch block in 15 and left ventricular aneurysm in 3. Our study, which is one of the largest with the longest postoperative follow-up period, documents that surgery results in good to excellent alleviation of symptoms in survivors. We currently recommend surgery for the symptomatic patient who has not responded to therapy with propranolol.     

Centenary of the first correct antemortem diagnosis of coronary thrombosis by Adam Hammer (1818--1878): English translation of the original report.

William Heberden (1710--1801), in 1768, described angina pectoris, the classic symptom of ischemic heart disease, 150 years after the discovery of the coronary circulation by William Harvey (1578-1657). Another 110 years had elapsed before the first antemortem diagnosis (confirmed at autopsy) of coronary thrombosis was reported by Adam Hammer in 1878. The patient was a 34 year old man who died some 19 hours after a sudden collapse. Although the patient's clinical features were atypical (such as the absence of angina and the presence of complete heart block) and the autopsy showed vegetative aortic endocarditis that appeared to be causally related to the thrombotic coronary occlusion, Hammer's astute and carefully reasoned bedside diagnosis was history-making and deserves to be so recognized.     

Tricuspid and pulmonary valve obstruction relieved by removal of a myxoma of the right ventricle

The first case of spontaneous and permanent complete heart block in a patient with well documented muscular subaortic stenosis is reported. Hemodynamic observations were made during the periods of complete heart block and paced rhythm. The exact cause of the complete heart block in this patient is uncertain.     

Reduction of intimal thickening in canine coronary bypass vein grafts with dipyridamole and aspirin

The potential benefit of platelet inhibitor drugs on coronary arterial bypass vein grafts was assessed in dogs with magnification-corrected angiographic luminal measurements and quantitative histologic evaluation of the vein grafts. There were 11 control animals and 11 animals treated with dipyridamole, 55 mg/day, plus aspirin, 325 mg/day. Eighteen animals with patent grafts were studied when electively killed 2, 4 or 6 months after grafting. At 14 days, there was greater angiographic narrowing in the most distal 1 cm of vein grafts in control than in treated dogs (P < 0.01). This same angiographic narrowing persisted in control dogs until they were killed (P < 0.03). Computer-assisted measurements of the entire area of intimal thickening were done on vein graft cross sections taken 1 cm from the distal anastomosis. The circumference of the vein grafts at the intimal-media junction was measured from the same section and the potential maximal luminal area calculated. The calculated luminal narrowing due to intimal thickening was greater in control than in treated dogs (P < 0.03). These data correlate well with the demonstrated angiographic narrowing. The findings indicate that the degree of early intimal thickening that persists 2 to 6 months postoperatively in canine coronary bypass vein grafts may be reduced by the platelet inhibitor combination of dipyridamole plus aspirin.     

Reduction of thrombosis in canine coronary bypass vein grafts with dipyridamole and aspirin

The potential benefit of platelet inhibitor drugs in reducing early thrombosis of coronary arterial vein bypass grafts was assessed in dogs. There were 26 control dogs and 24 dogs treated with dipyridamole, 55 mg/day plus aspirin, 325 mg/day. The dogs in both groups were killed at 2 hours and 1, 2, 3, 7 and 14 days after operation. The grafts were perfused with flxative in vivo, harvested and examined with light microscopy. Severe alterations of the graft wall were observed in the dogs in both groups. The grafts in the control group had a high incidence rate of thrombosis, which occurred early after the operation; those in the treated group had a significantly reduced incidence of thrombosis (p = 0.025). Our study indicates that a combined regimen of dipyridamole and aspirin is effective in reducing early graft thrombosis in dogs.     

Anatomy of coronary arteries in univentricular hearts and its surgical implications.

The coronary arterial anatomy in 26 univentricular hearts, its relation to the morphologic characteristics of the ventricles and rudimentary chambers, and its surgical implications were analyzed. All of the hearts except two had been operated on; 18 had septation with or without an extracardiac conduit and 6 had had palliative procedures. Twenty-one univentricular hearts with a left ventricular type main chamber had an anterior outlet chamber (17 left-sided subaortic, 3 right-sided subaortic and 1 right-sided subpulmonary). Right and left delimiting arteries outlined the outlet chamber in 16 hearts (76 percent). In 20 of the 21 hearts, large delimiting parallel branches of the right coronary artery course over the anterior wall of the heart; 13 of these vessels had been injured surgically with resultant ischemic myocardial necrosis. Five univentricular hearts did not have an outlet chamber; two had a left ventricular type main chamber and three had a morphologically right ventricular main chamber. Three of the five hearts had rudimentary pouches, located anteriorly in one and posteriorly in two. The two rudimentary pouches lying posteriorly were not outlined by delimiting arteries. Two of the five univentricular hearts without an outlet chamber also had injured coronary arteries. Thus, the identification of outlet chambers and rudimentary pouches in univentricular hearts is facilitated by the determination of coronary anatomy. The presence of major delimiting parallel branches over the usually favored ventriculotomy sites renders them vulnerable to surgical injury; such mishaps occurred in 15 of the 24 hearts that had either corrective or palliative operations.     

Effects of tocainide on ventricular fibrillation threshold. Comparison with lidocaine

In truncus arteriosus communis, as in other anomalies of conotruncal development, the coronary arterial anatomy not only differs from that found in the normal heart but also is subject to unpredictable variations. A consistently distinctive pattern was found in roughly two thirds of 31 cases, a frequency approximating that with which a distinctive coronary arterial pattern occurs in complete transposition of the great vessels. Surgical injury to a major coronary artery was identified in 2 of the 30 hearts from patients with truncus arteriosus communis who died in the early postoperative period. Of particular significance are large branches of the right coronary artery crossing the upper anterior surface of the right ventricle to supply the anterobasal surface of both ventricles and the upper part of the interventricular septum. These arteries are at special risk in surgical procedures utilizing a conduit anastomosed to a right ventriculotomy.     

Idiopathic dilated cardiomyopathy in the young: clinical profile and natural history

The clinical profile and course of documented cases of idiopathic dilated cardiomyopathy in children have been poorly characterized. Twenty-four patients (median age 2 years, range less than 1 month to 18 years) with idiopathic dilated cardiomyopathy were identified from Mayo Clinic records from 1973 to 1982. The most common presentation was congestive heart failure (92% of patients). Echocardiography (22 patients) generally revealed a dilated left ventricle with reduced fractional shortening (mean 14%) and ejection fraction (mean 26%). Two-dimensional echocardiographic evidence of left ventricular thrombus was present in 3 (23%) of 13 patients. Median cardiac index and left ventricular end-diastolic pressure (19 patients) were 2.5 liters/min per m2 and 22 mm Hg, respectively. Myocardial biopsy in eight patients showed nonspecific findings without active inflammation or evidence of endocardial fibroelastosis. On follow-up (mean duration 33 months, range 0 to 149), systemic arterial embolism had occurred in 2 (8%) of 24 patients. Fifteen of 24 patients had died (63% survival at 1 year and 34% survival at 5 years of follow-up). The cause of death was congestive heart failure in 11, complications after cardiac transplantation in 3 and sudden cardiac death in 1. Nine patients are alive at a mean follow-up time of 65 months (range 26 to 149); five are asymptomatic. Serial determination of left ventricular systolic function, available in all survivors, showed improvement in six patients and no significant change in three. Severe mitral insufficiency was present only in patients who ultimately died. A recent viral syndrome was noted more frequently in patients who survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hypertrophic obstructive cardiomyopathy and lentiginosis: A little known neural ectodermal syndrome

Eleven patients, 10 male, with classic hypertrophic obstructive cardiomyopathy and lentiginosis are described. Physical examination showed differences from the few previously reported cases in that (1) this condition was not confined to children; (2) mental retardation, sensorineural deafness and gonadal and somatic infantilism were either rare or absent; and (3) detailed family studies provided no evidence that this condition was inherited. Nine patients underwent cardiac catheterization and left ventricular angiography; all had left ventricular outflow obstruction and three had concomitant right ventricular outflow obstruction with a pressure gradient in excess of 100 mm Hg. Ten of the 11 patients were severely symptomatic, and 7, each with a left ventricular pressure gradient of more than 70 mm Hg, underwent successful septal myotomy/myectomy that resulted in marked symptomatic improvement that was maintained after long-term follow-up.     

Concealed retrograde bypass tracts and enhanced atrioventricular nodal conduction: An unusual subset of patients with refractory paroxysmal supraventricular tachycardia

The clinical and electrophysiologic features of eight patients with unusually rapid, medically refractory paroxysmal supraventricular tachycardia are described. Exercise induction of tachycardia and functional bundle branch block patterns during tachycardia were common. Tachycardia resulted from anterograde enhanced atrioventricular nodal conduction combined with retrograde conduction by a concealed left atrial-left ventricular accessory pathway producing rates ranging from 200 to 300 beats/min. Management and late follow-up study were characterized by generally unsuccessful electrophysiologic-pharmacologic testing and inconsistent rhythm control with continued drug therapy. Three patients underwent successful surgical interruption of the concealed accessory pathway, with elimination of recurrent tachycardias. These patients represent a unique subgroup with an identifiable electrophysiologic basis for unusually rapid tachycardias, potentially benefiting from invasive study and aggressive therapy.     

Communication between right pulmonary artery and left atrium

The 12th case of a direct communication between the right pulmonary artery and the left atrium is reported, with special emphasis on the clinical and hemodynamic findings. The anatomic differences in the 12 cases are categorized into three general types: an anomalous vessel connecting the posterior aspect of the right pulmonary artery and draining into the left atrium with (1) normal pulmonary venous connections or (2) the pulmonary vein draining into the anomalous vessel; and (3) an anomalous communication draining into the left atrium in place of the absent right lower pulmonary vein. In patients with this anomaly, surgery should result in complete cure; early intervention is recommended because the mortality rate is higher for patients who are older at operation and because there is an increased chance that cerebral and Systemic emboli will occur if surgery is not performed.     

Return to work after coronary angioplasty: A report from the national heart, lung, and blood institute percutaneous transluminal coronary angioplasty registry

Employment status was analyzed in 2,250 patients enrolled at 65 clinical centers in the NHLBI PTCA Registry. Patients were classified into 3 groups depending on the outcome of PTCA. In 63.6%, PTCA was successful without MI or CABG (Group A); in 25.3%, PTCA was unsuccessful and was followed by CABG (Group B); and in 11.1%, PTCA was unsuccessful and was followed by medical therapy alone (Group C). At entry, 68.3% of all patients were employed full- or part-time. The clinical characteristics of the 3 groups were different. Patients in Group C had a higher incidence of previous MI and previous CABG. In addition, patients in Group C had a significantly decreased baseline employment rate compared with those in Group A. At a mean follow-up of 1.5 years, there was a small but similar decrease in the percentage employed fullor part-time in all groups.

Employment status also was analyzed in a subset of 1,150 patients working full- or part-time at baseline and aged 60 years or younger, who would be expected to have the highest return to work rates. At a mean follow-up of 1.4 years, 81 to 86% of patients remained working irrespective of the outcome of PTCA. However, patients with successful PTCA returned to work significantly sooner. The occurrence of chest pain during follow-up in these patients was an important predictor of return to work, irrespective of the outcome of dilatation. In patients with chest pain during follow-up, only 77% were working, compared with 90% of patients who had not had chest pain.     

Two-dimensional echocardiographic assessment of dextrocardia: A segmental approach

Two-dimensional echocardiography was used in the prospective evaluation of 40 patients with the clinical diagnosis of dextrocardia. A segmental analysis of the situs, connections, ventricular anatomy, and chamber positions was utilized for a complete diagnostic assessment. An adequate examination was possible in 33 of these patients; the findings were confirmed by cardiac catheterization and angiography in 31 patients and at operation in 26.

Use of the location of the liver and the drainage of the hepatic veins and inferior vena cava allowed atrial visceral situs to be defined in 33 patients (solitus 21, inversus 9, and ambiguous 3). Pulmonary venous connections were correctly identified in 27. In 33 patients, atrioventricular (AV) and ventriculoarterial connections and ventricular anatomy were correctly predicted. Twenty patients had 2 separate well-developed ventricles. Ventriculoarterial connections were determined correctly in all 20 patients: concordant in 5, discordant in 6, double-outlet right ventricle in 5, and single-outlet right ventricle (pulmonary atresia) in 4. In 16 patients a ventricular septal defect was correctly identified. In the remainder the ventricular septum was intact.

Thirteen patients had univentricular heart: 8 had 2 AV valves (double-inlet ventricle), 3 had common AV inlet, and 2 had atresia of 1 AV connection.

Two-dimensional echocardiography allowed the accurate assessment of complex congenital heart defects associated with dextrocardia. Utilizing a segmental approach, one can correctly predict atrial-visceral situs, ventricular morphology and situs, and AV and ventriculoarterial connections.     

Cholesterol pericarditis terminating as constrictive calcific pericarditis: Follow-up study of patient with 40 year history of disease

This is a follow-up report of a patient with idiopathic cholesterol pericarditis (previously described in this Journal in 1961). The patient had had recurrent pericardial effusions since 1939, and died with calcific constrictive pericarditis some 40 years later. In the interim, the patient had an extended period (17 years) of symptomatic relief after pericardiotomy in 1958.