Pulmonary vascular disease in complete atrioventricular canal defect.

The incidence and time of occurrence of pulmonary vascular disease were studied in 67 children with the complete form of atrioventricular (A-V) canal defect. Advanced pulmonary vascular disease begins to develop during the first year of life, with intimal fibrosis (grade 3 cahnges) noted between age 6 months to 1 year. Vascular dilatation with plexiform lesions (grade 4 changes) can be found by age 1 year. These changes can be found in some patients in spite of hemodynamic findings usually considered to indicate lesser degrees of vascular disease. After age 2 years advanced pulmonary vascular disease is commonly found and may persist after surgical correction of the defects. A similar rapid progression of pulmonary vascular disease was noted in 40 children who had a large ventricular septal defect without A-V canal in whom systemic pressure was transmitted directly to the pulmonary vascular bed. Thirty-six of the 67 children had trisomy-21. No difference was noted in the speed of progression of pulmonary vascular disease between these children and those without trisomy 21. Palliative or corrective surgery should be performed in these patients by age 1 year to prevent development of advanced pulmonary vascular disease.     

Pulmonary vascular disease in complete transposition of the great arteries: A study of 200 patients

Lung specimens of 200 patients with transposition of the great arteries were examined microscopically for evidence of pulmonary vascular disease. In patients with an intact ventricular septum or a small ventricular septal defect, advanced pulmonary vascular disease was uncommon; only 9 of 107 such patients (8.4 percent) demonstrated greater than grade 2 (Heath-Edwards) pulmonary vascular disease. A persistent large patent ductus arteriosus appeared to promote progressive pulmonary vascular disease in this group since each of the five infants less than 1 year of age with grade 3 or 4 disease had this lesion. In contrast, pulmonary vascular disease was common in patients with a large ventricular septal defect; 37 of 93 patients (40 percent) with this defect had greater than grade 2 pulmonary vascular disease. Among patients more than 1 year of age, 26 of 35 (75 percent) had grade 4 disease. The catheterization data suggest that the calculated pulmonary vascular resistance may underestimate the degree of disease, probably by overestimating the pulmonary blood flow (Fick method). Pulmonic stenosis appeared to protect the lungs from progressive pulmonary vascular disease, and pulmonary arterial banding was protective when performed before age 6 months. Our studies indicate that a persistent large patent ductus arteriosus should be closed as early as possible in view of its association with advanced pulmonary vascular disease in these patients. In infants with a large ventricular septal defect, pulmonary arterial banding or corrective surgery with closure of the defect should be performed between the ages of 4 and 6 months to prevent progressive pulmonary vascular damage.     

Discrete subvalvular aortic stenosis in childhood: Study of 51 patients

Fifty-one children with discrete subvalvular aortic stenosis were studied between 1951 and 1974. The three anatomic types of obstruction found were the thin membranous type (43 cases), the fibromuscular collar type (5 cases) and the tunnel type (3 cases). The obstruction was usually severe, and the median left ventricular to aortic systolic pressure gradient was 90 mm Hg. Progressive obstruction with an increasing gradient was documented in 10 patients by serial cardiac catheterizations. Significant associated cardiac defects, present in 57 percent of patients, often masked the typical clinical and cardiac catheterization features of subaortic stenosis. The stenosis was often not discovered until after surgery for the associated defect.Forty patients underwent surgical resection of the discrete subaortic obstruction. After surgery significant left ventricular to aortic pressure gradients can be found at postoperative cardiac catheterization. These gradients may reflect inadequate resection of the more complex discrete obstructions or represent proliferation and regrowth of the previously resected subvalvular fibrous tissue. The criteria for operability of discrete subaortic stenosis should be the angiographic demonstration of a discrete subvalvular diaphragm and the presence of a resting left ventricular to aortic systolic pressure gradient of 40 mm Hg or more.     

Positional abnormalities of atrioventricular valves in transposition of the great arteries including double outlet right ventricle, atrioventricular valve straddling and malattachment.

Positional abnormalities (straddling or overriding) of the atrioventricular (A-V) valves were studied using angiographic, sector scan echocardiographic and postmortem anatomic data in 10 patients with transposition of the great arteries including double outlet right ventricle. Group I included six patients with tricuspid valve abnormalities. This group was further classified into: (a) patients with anular straddling and abnormal attachment of portions of the tricuspid leaflets in the left ventricle (malattachment) or on the ventricular septum, or both (four patients with d-transposition of the great arteries and hypoplastic right ventricle); and (2) patients with malattachment of portions of the tricuspid valve leaflet to the crest or left ventricular surface of the septum (septal malattachment) without significant anular straddling (two patients with d-transposition of the great arteries).Group II included four patients with mitral valve abnormalities without significant anular straddling, further classified into: (1) patients with septal malattachment of the portions of the mitral valve to the crest and right ventricular surface of the ventricular septum (two patients with double outlet right ventricle); and (2) patients with ventricular malattachment of portions of the mitral valve leaflets to the papillary muscles in the right ventricle (two patients with ventricular septal defect and discordant criss-cross atrioventricular connections).On the basis of these observations and reported experience, two main types of positional abnormalities of the A-V valves were recognized: (1) anular straddling in which the A-V anulus straddled the ventricular septum above the contralateral ventricle, together with ventricular leaflet malattachments, and (2) leaflet malattachment of either the septal or ventricular type without significant straddling of the valve anulus. Angiocardiography and sector scan echocardiography helped to identify these abnormalities of the A-V valves.     

Echocardiographic features of supracristal ventricular septal defect with prolapsed aortic valve leaflet.

Anatomically diagnostic echocardiographic features of a supracristal ventricular septal defect with prolapsed right coronary aortic leaflet are described in four children aged 2 to 10 years. Both single crystal M mode as well as 80 ° phased array sector scan techniques were used. The echographic features in the M mode scan from the aorta to the left ventricle in three of four patients included (1) the position of the ventricular septal defect as a clear space between the interrupted septal echoes below the aortic root, and (2) the prolapsed right coronary aortic leaflet as anomalous linear echoes in the right ventricular outflow tract. Angiographic, intraoperative and echocardiographic contrast studies were used to establish the diagnosis.On sector scanning using the long axis view, the supracristal ventricular septal defect was recognized as a clear space between the top of the ventricular septum and the anterior segment of the aortic root in three of four patients. The right coronary aortic leaflet was seen to prolapse into the right ventricular outflow tract through this defect, and its motion could be clearly followed during systole and diastole. It is concluded that echocardiography provides anatomic diagnosis of this lesion. Furthermore, the severity and progression of this lesion can be assessed by quantitation of the left ventricular size and performance.     

Two dimensional echocardiography in evaluation of right atrial masses: five cases in pediatric patients

Five unusual cases of a right atrial mass in children are described to illustrate the very valuable contribution that two dimensional echocardiographic examinations can bring to both the initial diagnosis and the subsequent management of patients with these findings. One patient had a large benign hemangioendothelioma of the right atrium. Two infants had extension of a Wilms' tumor from the kidney by way of the inferior vena cava to the right atrium. A fourth patient, an 8 year old girl, had no cardiac disease, and manifested Staphylococcus aureus endocarditis of the tricuspid valve with a large pedunculated mass and subsequent pulmonary embolus. A fifth patient, a premature infant with a central hyperalimentation catheter in the right atrium, had a large thrombus on the catheter that was successfully eradicated with urokinase-induced thrombolysis. Two dimensional echocardiography provides real time imaging of the entire right atrium, interatrial septum, inferior and superior venae cavae and tricuspid orifice and hence is valuable in the diagnosis and management of these clinical problems.     

Clinical manifestations of dynamic left ventricular outflow tract stenosis in infants with d-transposition of the great arteries with intact ventricular septum

Four infants with d-transposition of the great arteries and intact ventricular septum who manifested early clinical symptoms and deterioration due to dynamic left ventricular outflow stenosis are presented. All four had an anatomically adequate atrial septal defect, made at the initial balloon atrial septostomy, that was later confirmed intraoperatively. Two infants continued to have a low arterial oxygen saturation level because of inadequate interatrial mixing, and one of these had severe persistent cyanosis and was treated with the Mustard operation at age 4 days. The other two infants subsequently presented with hypercyanotic spells at age 3 months. All four infants had features of dynamic left ventricular outflow stenosis on hemodynamic, angiocardiographic and echocardiographic studies. The left ventricular outflow pressure gradient was shown to increase after administration of isoproterenol in one infant, and relief of a cyanotic spell with reduction of left ventricular systolic pressure was achieved in another after intravenous administration of propranolol. The Mustard operation relieved symptoms in all infants. The effect of left ventricular outflow tract stenosis on the mechanisms responsible for interatrial mixing in d-transposition of the great arteries with intact ventricular septum is discussed.     

Masked subaortic stenosis in ostium primum atrial septal defect: Recognition and treatment

Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic “goose-neck” deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.     

Echocardiographic assessment of discrete subaortic stenosis in childhood

The validity of Laplace's relation for assessing peak left ventricular pressure has previously been demonstrated in children with congenital aortic valve $\text{s}$tenosis by using the echocardiographically determined end-diastolic $\text{h}\text{r}$ ratio (that is, the ratio of mean left ventricular septal and posterior wall thicknesses to half the minor left ventricular cavity axis). This report examines the applicability of this ratio to the preoperatlve assessment of left ventricular pressure in 17 children with discrete subaortic stenosis. Because the regression equation derived from previously published data on patients with aortic valve stenosis and the present data on children with subaortic stenosis were similar, a pooled estimate of peak left ventricular pressure (LVP) in children $\text{w}$ith left ventricu$\text{l}$ar outflow tract obstruction is presented: $\text{LVP}\text{= 312.23 (}\text{h}\text{r}\text{) ± 28.01 (}\text{h}\text{r}\text{)}$.Additionally, the left ventricular outflow tract was measured as the anteroposterior dimension between the first recognizable closure point of the mitral valve and the interventricular septum and expressed as the left ventricular outflow tract/aortic root dimension ($\text{LVOT}\text{Ao}$) ratio. This ratio was tower in patients with subaortic stenosis than in control subjects and was helpful in differentiating patients with mild stenosis from those with more severe stenosis. The prevalence of echocardiographically observed features such as early systolic closure of the aortic valve and fluttering of the aortic valve leaflets could not be correl$\text{a}$ted with the severity of stenosis. It is concluded that the end-diastolic $\text{h}\text{r}$ ratio and the $\text{VOT}\text{Ao}$ ratio are quantitatively useful in the assessment of subaortic stenosis, whereas the commonly present motion abnormalities of the aortic valve are not indicative of the severity of the subaortic lesion.     

Prognostic significance of arrhythmia in tetralogy of fallot after intracardiac repair

The significance of arrhythmia and conduction defects was studied in 395 patients who underwent intracardiac repair of tetralogy of Fallot between 1958 and 1976. In 91 of these patients (group I), the median date of repair was 1964 and all underwent stress testing. In 107 patients (group II), the median date of repair was significantly later (1971), but all subjects were too young for stress testing. The remaining 197 patients (group III, median date of repair 1963) included all those who did not participate in stress testing or who had died; 42 of these patients were later excluded from analysis because of insufficient electrocardiographic records. The overall incidence of any form of arrhythmia (19 percent) was the same for each group. However, in group II (patients who underwent intracardiac repair more recently), no tachyarrhythmia was observed and there were fewer patients with 1° atrioventricular (A-V) block (p <0.001) and more patients with complete right bundle branch block (p <0.001) than in groups I and III. In two patients (both in group I), unexpected, late sudden death unrelated to strenuous physical activity occurred. Arrhythmia was least common in patients with primary intracardiac repair and more common in those with either palliation before intracardiac repair or multiple intracardiac repairs. In group I, 41 of 91 patients with arrhythmia of even minor degree at rest (n = 13) or exercise (n = 28) had a significantly larger heart, greater right ventricular pressure and lesser exercise performance than did those without arrhythmia, thus suggesting the presence of residual abnormality or dysfunction. No tachyarrhythmia was induced by exercise in this study. Patients with either arrhythmia or bifascicular block have generally had a benign course without strong evidence of progression of arrhythmia.     

Functional aortic valve atresia in transposition of the great arteries

The criterion for the diagnosis of functional atresia of a patient semilunar valve is met when the pressure in a ventricle remains lower than that in the related great artery throughout systole so that no forward flow can occur. Functional pulmonary valve atresia has been well recognized in infants with normally related great arteries and massive tricuspid valve incompetence. The cardiac physiology and anatomy of an infant with transposed great arteries and functional aortic valve atresia is reported for the first time. The peak systolic pressure in the right ventricle was 30 mm Hg and in the aorta 64 mm Hg. The causes for right ventricular incompetence were abnormalities of the tricuspid valve and hypoplasia of the ventricular free wall. Three other cases with similar ventricular anatomy and physiology but with anatomic atresia of the aortic valve are reviewed. The possibility that under these physiologic circumstances during fetal life functional atresia develops first, and that anatomic fusion of idle semilunar cusps develops as a secondary phenomenon, is discussed.     

Two dimensional echocardiographic evaluation of mustard operation for d-transposition of the great arteries

Two dimensional sector scan echocardiography was used to evaluate the morphologic characteristics of the surgically revised atria in 17 patients with d-transposition of the great arteries who had undergone the Mustard operation. Echocardiographic imaging of the atria was obtained from various planar projections. Dimensional measurements of various segments of the systemic and pulmonary venous atria were obtained in each patient. Correlative hemodynamic, angiographic, postmortem and echocardiographic data showed that seven patients (Group I) had no structural abnormalities of the atria. These 7 patients served as controls for 10 other patients with structural abnormalities of the surgically created atria. One patient (Group II) showed stenosis of the junction of the superior vena cava and systemic venous atrium compared with findings in the control group. Three patients (Group III) had significantly reduced echocardiographic dimensions of the junction of the anterior and posterior segments of the pulmonary venous atrium. Six patients (Group IV) had increased echocardiographic dimensions of all components of the pulmonary venous atrium due to tricuspid regurgitation. These data show that qualitative and quantitative two dimensional sector echocardiography can reliably detect structural abnormalities of the surgically revised atria after the Mustard operation.     

Distal aortopulmonary septal defect,aortic origin of the right pulmonary artery,intact ventricular septum,patent ductus arteriosus and hypoplasia of the aortic isthmus: A newly recognized syndrome

The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and Interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to “steal” from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.     

Two-dimensional echocardiographic prospective diagnosis of common truncus arteriosus in infants

Two-dimensional echocardiographic prospective diagnosis of truncus arteriosus was made in 7 infants. Two infants had truncus arteriosus type I, 3 patients had truncus arteriosus type II, 1 infant had truncal valve stenosis with an interrupted aortic arch, and 1 had type IV truncus arteriosus with pulmonary hypertension. Multiple imaging views were utilized to confirm the diagnosis. The parasternal long-axis view demonstrated the great vessel-ventricular septal override and the origin of the pulmonary arteries from the posterior aspect of the ascending truncus. The suprasternal notch image facilitated identification of the left- or right-sided aortic arch and the origin of the pulmonary arteries from the truncus. Transverse imaging sections at the base of the heart facilitated identification of the pulmonary artery origin of truncus arteriosus type I. Subcostal coronal and sagittal views imaged the common truncus and the ventricular septal defect.

These echocardiographic images were contrasted with and discriminated from those of an infant with aorticopulmonary window with intact ventricular septum. Although cardiac catheterization and angiography may be required to assess pulmonary arterial pressure, pulmonary vascular resistance, and the distal pulmonary arterial anatomy in truncus arteriosus, 2-dimensional echocardiography can be used to correctly establish the morphologic diagnosis of truncus arteriosus in infants.     

Two-dimensional echocardiographic features of interruption of the aortic arch

The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized from an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.     

Aortocoronary bypass surgery: Correlation of angiographic symptomatic and functional improvement at 1 year.

Angiographic changes in the coronary circulation were evaluated in 60 patients 1 year after aortocoronary bypass surgery, and their relation to the postoperative clinical status was examined. Of 124 grafts implanted, 26 were closed, 7 stenotic and 91 (74 percent) patent at 1 year. Progression of occlusive disease occurred in 21 of 57 (37 percent) nongrafted and 78 of 123 (63 percent) grafted vessels. On the basis of location and severity of progression, significant lesions bypassed and patency of grafts, postoperative coronary perfusion was considered optimal in 16 patients (Group I), better in 24 (Group III). Complete freedom from chest pain or lessening of pain (improvement by two New York Heart Association functional classes) occurred in 88 and 79 percent of patients in Group III. Positive preoperative treadmill stress tests became negative after surgery in five of six patients in Group I, five of eight in Grojp II and three of eight in Group III. This study demonstrates that when progression of disease, graft patency and extent of revasculariztion are considered in combination, the postoperative angiographic status of the coronary circulation correlates well with clinical improvement at 1 year. These findings support the hypothesis that improved blood supply to ischemic myocardium is a major factor contributing to relief of angina pectoris after saphenous vein bypass surgery.