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Sudden, unexpected cardiac death in the age group 1 to 21 years usually is due to myocarditis, hypertrophic cardiomyopathy, aortic valvar stenosis, and coronary arterial abnormalities. The hearts of 70 patients <21 years of age who died suddenly were reviewed. Twenty patients were <1 year of age and 50 were 1 to 21 years old. The cardiac findings were compared with those in 68 age-matched controls with known cardiac disease who did not die suddenly. Significant cardiac abnormalities were present in 13 (65%) of the 20 infants; 10 (50%) had anomalies of the aortic origin of the coronary arteries. Among the 50 older patients, cardiac abnormalities were found in 40 (80%), among whom coronary arterial anomalies existed in 12 (24%). Anomalies of aortic origin more frequently involved the left main than the right coronary artery in both groups.

This study investigated the effect of anisodamine (2 and 5 mg/kg i.v.) on ocular and systemic blood flow distribution in awake lambs using the radioactive microsphere technique. In separate in vitro studies, the effects of anisodamine (at final concentrations of 0.01 to 2.5 mg/ml) were determined on arachidonic acid, alloxan and ultraviolet radiation-induced lipid peroxidation of isolated retinal cells from rabbits and on alloxan-induced lipid peroxidation of hamster pancreatic islet beta cells. Malondialdehyde production was used as an index of lipid peroxidation and measured by the thiobarbituric acid method. Anisodamine preferentially increased blood flow and oxygen delivery to the retina-choroid and iris-ciliary body of the eye by 50-100%. Anisodamine significantly attenuated lipid peroxidation in retinal cells induced by ultraviolet radiation, alloxan and arachidonic acid by 17-50% and protected pancreatic beta cells against alloxan-induced lipid peroxidation. These properties may, in part, account for the beneficial effect of anisodamine in certain patients with diabetes.

A review of 18 cases with discrete subaortic stenosis (DSS) revealed that involvement of the aortic valve cusps in the basic process was present in 16. In these 16 cases, extensions of fibroelastic tissue from the site of the DSS toward the base or superior to the base of one or more cusps were seen. In seven of the involved cases, deformity of a cusp was associated with these extensions. Valvular aortic insufficiency in cases with DSS might be caused by extensions of the fibroelastic tissue to the aortic cusps.

Children with acyanotic congenital heart disease frequently develop respiratory difficulties such as atelectasis, pneumonia, or infantile lobar emphysema. In some cases, the cause of the respiratory difficulty is compression of the tracheobronchial tree by hypertensive dilated pulmonary arteries, since this type of heart disease frequently demonstrates large left-to-right intracardiac shunts. Sites of predilection for compression include the left main bronchus, the left upper lobe bronchus, the junction of the right bronchus intermedius and right middle lobe bronchus, and the left side of the distal trachea. Cardiac anomalies which predispose to this type of compression include ventricular septal defect, patent ductus arteriosus, interruption of the aortic arch, and tetralogy of Fallot. Pulmonary arteriopexy may relieve the tracheobronchial compression.

Among 49 heart specimens with total anomalous pulmonary venous connection (TAPVC), obstruction to pulmonary venous flow was present in all 13 cases with TAPVC below the diaphragm and in 53% of 36 cases with TAPVC above the diaphragm. Obstruction was produced by extrinsic pressures on the vein, intrinsic narrowing of the vein, or both. The histology of the narrowed veins was extremely variable, ranging from atrophy of the vein wall to hypertrophy of intima, media-adventitia, or both. Balloon dilation of narrowed veins was performed in 3 cases, without clinical or anatomic evidence of success.

Four neonates with scimitar syndrome are presented. Three of the cases had congenital cardiovascular abnormalities not usually regarded as part of the scimitar syndrome, namely, ventricular septal defect, abnormalities of the aortic arch, and abnormal relationship of the pulmonary arteries and bronchi. Review of the literature indicates that among subjects of all ages the incidence of additional congenital heart disease is 24%; in patients within the pediatric age group the incidence is about 36% and is highest (75%) among those subjects having diagnostic studies while neonates.

Our study was undertaken to determine the type, incidence, and functional significance of residual anomalies in patients who have undergone corrective repair for tetralogy of Fallot. We reviewed data from cardiac catheterisations performed on 132 survivors. A significant residual ventricular septal defect was present in only 12 patients. Resting right ventricular systolic pressure was less than 80 mmHg in 100 patients and ranged from 80 to 150 mmHg in the other 32 patients. Thirty-five patients were studied both at rest and during supine exercise. In most patients, the relation between oxygen consumption and cardiac output was normal during exercise. The stroke index and right ventricular end-diastolic pressure at rest and on exercise were compared in 34 patients. Seventeen showed a normal response to exercise. In the other 17 patients, right ventricular end-diastolic pressure rose on exercise; in 5 of these the stroke index fell during exercise, indicating abnormal myocardial response. Our studies indicate the frequent occurrence of residual abnormalities, even in patients who appear asymptomatic, after total correction of tetralogy of Fallot.

Sixteen consecutive patients (12 with tricuspid atresia) underwent a Fontan procedure, with no operative deaths. There were three late deaths. Eleven of the survivors were electively catheterized 4-25 months postoperatively. Rest and exercise hemodynamics were measured in five patients, and resting hemodynamics alone were measured in three. All exercised patients were New York Heart Association class I Cardiac index was low at rest (2.3 +/- 0.61/min/m2) and during exercise (4.9 +/- 1.11/min/m2) due to a low stroke index both at rest (28 ml) and exercise (35 ml). Accordingly, mixed venous oxygen saturations were decreased (66% at rest and 31% during exercise). These values are significantly lower than those at rest and during exercise from 23 control patients of similar age and size. Heart rates, pulmonary vascular resistances, and left ventricular filling pressures appeared normal both at rest and during exercise. High right atrial pressure at rest (15 mm Hg) was associated with minimal conduit gradient (2 mm Hg). However, exercise increased the mean conduit gradient to 8 mm Hg, demonstrating significant functional conduit obstruction. As expected, the cardiovascular response to exercise is abnormal after the Fontan procedure, even in asymptomatic patients. This abnormal response may be exacerbated by conduit obstruction, and conduit obstruction may not be apparent during resting studies.

Twenty cases of cor triatriatum are reported. The diagnosis was confirmed by necropsy in 16 cases and at the time of operation in 4. The lesion occurred as an isolated anomaly in 7 cases; in 13, other associated cardiac anomalies were present. Three anatomic types of cor triatriatum were identified in the cases studied at necropsy: diaphragmatic (10 cases), hourglass (3) and tubular (3). The diaphragmatic type was also present in all four cases in which the diagnosis was confirmed at operation. Associated anomalies were found in five cases of the diaphragmatic type and in each case of the hourglass of tubular types. In isolated cor triatriatum the clinical findings were characteristic of pulmonary venous and arterial hypertension. In two cases, one with a communication between the right atrium and the accessory left atrial chamber and one with partial anomalous pulmonary venous connection associated with cor triatriatum, the clinical findings suggested a large left to right shunt with pulmonary arterial hypertension. The clinical findings varied in the cases with associated anomalies, and it was difficult to determine the cause of disturbance of the circulation.

Balloon dilation angioplasty was attempted in 10 infants and children with severe congenital or operative "venous" obstructions. In five children the obstructions were "vena caval" and followed repair for transposition of the great vessels (four patients) or orthotopic liver transplantation (one patient). In the four patients with fixed vena caval or baffle obstructions, balloon angioplasty was successful in relieving the obstruction, decreasing the average gradient (16.0 to 4.5 mm Hg), and increasing the average diameter (3.0 to 8.9 mm) of the obstructed site. One child died 1 week later from an unrelated cerebral hemorrhage; the three survivors have had persistent clinical and angiographic improvement. The fifth child had severe systolic narrowing of the superior baffle limb caused by marked tricuspid regurgitation, which ballooned the superior limb of the baffle against the atrial roof. Angioplasty was unsuccessful in relieving this type of obstruction, which resolved with tricuspid valve replacement. Of the five infants with obstructed pulmonary veins, three had congenitally narrowed vessels associated with total anomalous pulmonary venous connection, one had acquired stenosis, and one had postoperative obstruction after repair of a mixed type of total anomalous pulmonary venous connection. Dilation was unsuccessful in all five patients, but for different reasons: in the congenitally narrowed veins, the waist in the balloon could not be eliminated, even with high dilating pressures; in the infant with acquired stenosis, the vein stretched but did not tear at low dilating pressures; and in the postoperative obstruction, angioplasty increased the diameter of the obstruction but did not increase flow to the affected lung.(ABSTRACT TRUNCATED AT 250 WORDS)

Eighteen percent of heart specimens with isolated ventricular septal defect also had a floppy mitral valve. There was no statistical difference in the incidence of floppy mitral valve in the three age groups considered (less than 1 year, 1 to 16 years and 17 to 91 years). In no patient was a floppy mitral valve considered to be the cause of death. Complications of floppy mitral valve (ruptured chordae tendineae, bacterial endocarditis, mitral regurgitation and fibrin deposits at the mitral valve-left atrial angle) occurred at approximately the same frequency as that reported in autopsy studies of isolated floppy mitral valve. In the specimens with floppy mitral valve and ventricular septal defect, 63% also had floppiness of the tricuspid valve, 16% of the pulmonary valve and 5% of the aortic valve. The anatomic basis for floppy mitral valve was considered to be spongiosal invasion and disruption of the fibrosa of the valve leaflet. In this study, spongiosal invasion of the fibrosa was fully developed by 3 months of age and there was no evidence that the incidence or severity of spongiosal invasion increased between the ages of 3 months and 88 years. These data suggest that the floppy mitral valve is a congenital lesion that reaches full anatomic expression in infancy. No evidence was found that ventricular septal defect and floppy mitral valve share a common etiology.

The pathologic appearance of pulmonary arteries subjected to balloon dilation was studied in four subjects with stenosis of pulmonary arteries. Nine vessels were dilated. Successful dilation in seven vessels was accompanied by intimal disruption and tearing of the media. In one vessel, at the site of a previous surgical procedure, dilation could not be accomplished. Histologically, this vessel was encased by reactive fibrous tissue, which may have precluded successful dilation. In one case, simultaneous rupture of the dilating balloon and the left pulmonary artery occurred. Morphologic examination could not adequately explain the cause of vessel rupture. Among the six vessels successfully dilated and studied 4 to 14 months after the dilation, the postdilation luminal diamter had been maintained. Tears in the intima and media as seen histologically had been filled in by scar tissue. In one artery a dilated segment distal to a residual obstruction revealed marked intimal proliferation.

The pathological anatomy of 109 specimens of aortic valvular atresia was reviewed for the purpose of identifying the cardiovascular anomalies associated with that condition. We found the most commonly associated anomaly to be coarctation of the aorta, which was present in 71 percent of our cases and judged to be hemodynamic significance in one-third of the involved cases. Other associated anomalies, in order of decreasing frequency, were mitral atresia, anomalous systemic and pulmonary venous connections, abnormalities of branching of the aortic arch, and ventricular septal defect. The study demonstrated that aortic atresia is associated with a significant incidence of other cardiovascular anomalies. Additional anomalies, when present, may complicate emerging attempts at surgical correction of this condition.

Five cases are described in which noninflammatory pulmonary arterial aneurysmal disease existed. In each case, severe pulmonary hypertension had been present. In one (woman, age 50 years), the pulmonary hypertension was of the primary type, while in each of the others, congenital heart disease was an underlying condition (atrial septal defect in three cases; each woman, age range 35 to 56 years) and complete transposition in the remaining case (boy, age ten). In one case with atrial septal defect, saccular aneurysm involving each major pulmonary arterial branch was considered to have resulted from atherosclerotic disease. In each of the remaining cases, cystic medial necrosis was present, and the aneurysmal disease was the result of laceration of a major pulmonary arterial segment. In one of the cases with atrial septal defect, the laceration was recent and extended through the full thickness of the pulmonary trunk in a circumferential manner. In the remaining three cases, lacerations were usually old and had not extended through the full thickness of the involved arterial segments. Saccular aneurysm formation (usually multiple) had resulted from weakness of the wall as a result of laceration and retraction of tissue.

Nine children with isolated cleft mitral valve, aged 1 day to 12 years, were studied. The electrocardiogram showed a normal QRS axis in 5 subjects. Cardiac catheterization was performed in 4 patients and demonstrated severe mitral insufficiency in 3 but failed to clearly demonstrate a cleft mitral valve or gooseneck deformity. The mitral cleft was confirmed at operation in 2 patients. Two-dimensional echocardiography demonstrated a cleft dividing the anterior mitral leaflet into 2 portions in each patient. The mitral anulus was normally positioned and the atrioventricular septum present. Atrial and ventricular septa were intact. Features similar to anatomic studies such as accessory chordae and thickening of the edges of the cleft with increasing age were also seen. Two-dimensional echocardiography is the only method available to reliably diagnose isolated cleft of the mitral valve.

PDA was diagnosed in 76 (19%) of 396 patients with RDS. Evidence of excessive pulmonary blood flow and CHF developed in 15 (20%). CHF was not recognized prior to 10 days of age in any patient. All 15 with CHF were initially managed medically. Seven improved; three died of other causes. Five patients who failed medical management were surgically treated; all survived operation, but only two were improved.

Two patients had cardiac complications of childhood onset systemic lupus erythematosus (SLE). A 14-year-old boy had extramural and intramural coronary arteritis demonstrated by angiography. The signs and symptoms of myocardial ischemia improved dramatically when corticosteroid dose and immunosuppression were increased. A 20-year-old woman had had SLE for 18 years and had functionally significant mitral stenosis and regurgitation. Calcification of the mitral valve was observed on echocardiography. Corticosteroid treatment has controlled the renal manifestations of SLE. Cardiac complications of SLE are now a significant clinical problems.

An 11-year-old girl was found to have completely obstructed left pulmonary veins eight years following corrective surgery for transposition of the great arteries. The patient was acyanotic and asymptomatic. Retrograde flow of arterial blood from the affected left lung accounted for an angiographic appearance that mimicked occlusion of the left pulmonary artery and resulted from a failure of systemic venous development. Pulmonary venous anatomy could only be demonstrated by pulmonary arterial wedge angiographic studies. This experience emphasizes that complete unilateral pulmonary venous obstruction may occur in an asymptomatic patient and underlines the importance of investigating pulmonary venous anatomy in any patient with gross inequality of the distribution of pulmonary blood flow.

Three siblings evaluated for syncope were found to have left bundle-branch block. Progression to complete heart block occurred in all. Pacemaker implantation eliminated syncopal epidoses in each case. Echocardiographic manifestations of cardiomyopathy were present in each child despite normal roentgenographic heart size. Careful family study identified no other affected members with conduction defects. These children area believed to represent a form of familial cardiomyopathy in which clinical manifestations of cardiac conduction system disease predominate.

Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.

Twenty-two consecutive infants and children underwent aortopulmonary shunt operations with polytetrafluoroethylene grafts. Thirteen infants younger than 6 months of age (group I) received 14 grafts (4--5 mm in diameter, 0.5--2.5 cm long). Two shunts became obstructed, including kinking in the single 2.5-cm graft. Eight of the nine long-term survivors had pulmonary artery pressures measured a mean of 14 months postoperatively. In all infants, pulmonary resistance was normal and mean pulmonary artery pressure was less than or equal to 20 mm Hg, except in the infant with a 5-mm graft. In nine children 7 months to 15 years of age (group 2), the rate of graft occlusion was higher (five of nine). There was no evidence of pulmonary artery distortion secondary to shunt placement in either group. On late follow-up, blood hemoglobins were less than 20 g% as late as 30 months of age; acceptable palliation may be expected until at least 2 years of age and 12 kg in weight are reached. We conclude that short, 4-mm-diameter polytetrafluoroethylene grafts provide good palliation in infants with cyanotic congenital heart disease, with minimal risks from pulmonary hypertension or pulmonary artery distortion. Such grafts are less successful in older children, and should only be considered when other forms of surgical relief are not feasible.

Although the Heath-Edwards classification has been used for more than 25 years to evaluate pulmonary vascular changes, its potential to predict the long-term course after successful ventricular septal defect closure has not been proved. Operative lung biopsy slides obtained at the time of closure from 57 infants and children who had been among the first survivors (between 1954 and 1960) of such surgery were graded in blinded fashion according to the Heath-Edwards classification system, and the resultant biopsy grade of each was compared with the eventual long-term outcome of each child. In 53 (93%) of the 57 cases, the Heath-Edwards system correlated well with long-term clinical or hemodynamic status of the patient. Grade IV changes were predictive of a usually fatal outcome, while grade I and II changes were generally benign. Death from pulmonary vascular disease occurred, however, in four children whose operative lung biopsy had been classified as either grade I or II, indicating that the focal nature of the higher Heath-Edwards grades, or human error, must be considered when this classification system is employed.

Percutaneous transluminal balloon dilation angioplasty of an aortic coarctation was done in a one-day-old boy. The infant died 10 h later during aortic valvotomy. A dissecting aneurysm of the aortic arch was present at autopsy.

Adequate treatment of pulmonary artery compression of the tracheobronchial tree requires a high index of suspicion for the diagnosis, precise localization of the sites of airway compromise by bronchoscopy, and accurate identification of the anatomy of the obstructing vascular structures. Surgical correction of this vascular anomaly to relieve airway compression is necessary in many infants and should be performed promptly. Pulmonary artery plication, arteriopexy, or aneurysmorrhaphy is well tolerated and can be dramatically successful in improving airway patency.

To better determine the risks of transcatheter closure of a patent ductus arteriosus (PDA), a model of PDA was made in newborn piglets by using 5- to 7-mm angioplasty catheters to dilate the probe PDA. This maneuver resulted in a permanent PDA in most piglets. Four to 6 weeks later PDA closure was attempted using the Rashkind PDA occluder. Twelve such procedures were attempted, using clean but nonsterile technique. Nine of 12 PDAs were successfully closed. Two failures were the result of inability to successfully traverse the PDA. This problem was solved by using a long sheath to position the device properly. Four complications occurred, all related to device release: left pulmonary artery embolization in 1 case, femoral artery embolization in 1, torn pulmonic valve cusp in 1 and lodgment of a prosthesis on a pulmonic valve cusp. Two successfully implanted devices were infected at necropsy. This study demonstrates the value of a piglet model in testing transcatheter PDA occlusion devices, the importance of sterile technique in such procedures, the hazards of device retrieval through the right heart and the feasibility of transcatheter PDA closure.

The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract.

In three cases, two in children and one in a young adult, ventricular outflow obstruction was caused by a valvelike flap of accessory endocardial tissue. The angiocardiographic feature was that of a narrow radiolucent, crescent-shaped or linear filling defect corresponding to the site of obstruction. Resection of the accessory tissue was performed in each case at the time of correction of associated conditions. In one case (a woman 20 years of age) the obstructing membrane was in the outflow tract of the right ventricle, and a ventricular septal defect was associated. In each of the other two cases the outflow tract of the left ventricle was the site of obstruction. In one (a 14-month-old boy), an ostium primum type of atrial septal defect and cleft mitral valve were associated; in the remaining case (a 7-year-old boy) complete transposition and ventricular septal defect were also present. In the latter case a Mustard procedure was performed and was followed by death. Successful results were obtained in the first two patients.

The effect of carbon monoxide inhalation on the regional distribution of right and left ventricular myocardial blood flow was studied in 12 closed-chest anesthetized dogs. Dogs were exposed to a nonhypoxic mixture of oxygen (21-40%) and carbon monoxide (1.5-2.0%) for 10 min. Myocardial blood flow was measured (15 micron radionuclide-labeled spheres) during control conditions, and 10 and 60 min following discontinuation of carbon monoxide corresponding to carboxyhemoglobin levels (COHb) of 41.6 +/- 2.8 and 26.5 +/- 1.6% (mean +/- SE), respectively. At COHb level of 26.5%, right and left ventricular blood flows were increased to approximately 1.8-1.9 times the control values (1.06 +/- 0.10 vs 0.64 +/- 0.08 mL/min per gram and 1.72 +/- 0.12 vs. 0.91 +/- 0.07 mL/min per gram, respectively, P less than 0.002). At a COHb level of 41.6%, both right and left ventricular vascular beds were maximally or near maximally dilated as right ventricular and left ventricular myocardial blood flow values were increased approximately fivefold. The right and left ventricular subendocardial-subepicardial flow ratios were reduced at both COHb levels (P less than 0.05). Thus, in addition to the global myocardial hypoxia that occurs following elevation of the COHb level, relative subendocardial underperfusion is a component of carbon monoxide poisoning in the intact dog.