Search

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy-truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.

We report the first known case of supero-inferior ventricles with a superior morphologically left ventricle and an inferior morphologically right ventricle. This 2 1/2-year-old boy also had dextrocardia, double-outlet right ventricle [S,L,L], right-sided mitral atresia, left-sided tricuspid regurgitation, a large conoventricular type of ventricular septal defect, and pulmonary outflow tract stenosis. This very rare form of superoinferior ventricles appears to be due to excessive levorotation (approximately equal to 170 degrees) of discordant L-loop ventricles.

BACKGROUND Despite the current clinical use of radiofrequency (RF) catheter ablation in infants, the acute and late effects of RF lesion production in immature myocardium remain unknown. This study was specifically designed to investigate the pathology of RF lesions in developing sheep myocardium. METHODS AND RESULTS In study 1, RF lesions were made on the epicardial left ventricular surface of the beating heart in 15 sheep, 5 approximately 4 weeks of age (11.0 +/- 1.0 kg) and 10 approximately 8 weeks of age (23.8 +/- 3.4 kg), to assess the effects of RF application duration (10 to 90 seconds) and electrode tip temperature (45 degrees to 90 degrees C) on lesion size in immature myocardium. Lesion width and depth increased asymptotically with RF duration, to 7.0 +/- 0.7 and 4.8 +/- 1.0 mm at 90 seconds, respectively. The time to reach one-half lesion size was 6.5 seconds for width and 12.0 seconds for depth. Lesion width increased nearly linearly with tip temperature above 50 degrees C, but depth followed a sigmoid relation, with no increase above 80 degrees C. In study 2, RF lesions were made in all four cardiac chambers under fluoroscopic guidance in 19 infant sheep (10.9 +/- 1.4 kg). Lesion sizes and histological characteristics were assessed acutely (acute, n = 5), at 1.07 +/- 0.02 months (1 month, n = 5), and at 8.5 +/- 0.5 months (late, n = 9). Atrial and ventricular lesions but not atrioventricular groove lesions apparently increased in size during the follow-up period. Atrial lesions width increased from 5.3 +/- 0.5 to 8.7 +/- 0.7 mm at 1 month (164%) but did not increase further at late follow-up, while ventricular lesion width increased from 5.9 +/- 0.8 to 10.1 +/- 0.7 mm (171%) at late follow-up but was not significantly changed at 1 month. Histological evaluation revealed replacement of normal myocytes with fibrous and elastic tissue at 1 month and late follow-up in all locations but also demonstrated a poorly delineated border with multiple extensions of fibrous and elastic tissue into surrounding normal myocardium in late ventricular lesions. CONCLUSIONS RF lesion formation in immature sheep myocardium is similar to that in adult myocardium acutely but is associated with late lesion enlargement and fibrous tissue invasion of normal myocardium. These findings may have implications for clinical RF ablation procedures in infants.

Two cases of nonimmune hydrops fetalis and fetal death associated with cardiac rhabdomyoma are reported. Case 1 presented with fetal supraventricular tachycardia, and cardiac rhabdomyoma was accurately diagnosed by fetal echocardiography. Autopsy revealed multiple rhabdomyomata involving the right atrial free wall, the sinoatrial node, and the left ventricle. The left circumflex coronary artery was extrinsically compressed by adjacent tumor tissue, causing left ventricular myocardial infarction. Case 2 had a unique, pedunculated, ball-like rhabdomyoma that almost totally occluded the mitral orifice. The causes of fetal death in patients with cardiac rhabdomyoma are analyzed and the possibility of fetal surgical management is proposed. (Chest 1991; 99:139–43)

Since the posterior wall of the right superior vena cava (RSVC) is contiguous with the anterior wall of the right upper pulmonary veins, a localized defect in this common wall may create a cavopulmonary venous confluence without eliminating the normal connection of the same right pulmonary veins with the left atrium (LA). Through this defect, blood of the unroofed right pulmonary veins will drain into the RSVC and right atrium (RA), and blood from the RSVC may shunt into the right pulmonary veins and LA. Hemodynamically, the RSVC will become biatrial. If the RSVC blood flows preferentially into the LA, its right atrial orifice will become stenotic or even atretic. If atretic, the normally positioned RSVC will drain entirely into the LA. In this report, we present the clinical and anatomical findings of two postmortem cases with biatrial drainage of the RSVC. We also document the clinical, echocardiographic, angiocardiographic, and surgical data of a living patient with left atrial drainage of the RSVC and tetralogy of Fallot with pulmonary atresia. The relevant literature and surgical treatment are reviewed, and the morphogenesis of the biatrial and left atrial RSVC is considered.

We report the third known case of origin of the right and left pulmonary artery branches from the ascending aorta via a short common pulmonary artery. A large unbranching main pulmonary artery opened through a patent ductus arteriosus into the descending thoracic aorta. Preductal coarctation of the aorta and multiple congenital anomalies were also present. This rare cardiovascular malformation facilitates a new anatomic and developmental understanding of truncus arteriosus.

The STARFlex system is a modified CardioSEAL device with a flexible self-centering mechanism comprised of nitinol springs strung between opposing arms, a connecting ball (sleeve joint that allows the device to pivot prerelease), and a front-loading delivery system. It was designed to allow a smaller device/defect sizing ratio and delivery profile, provide centering capability, and improve closure rates. To test this system, 13 devices (23, 28, and 33 mm) were deployed in six sheep within created atrial septal defects (12- to 22-mm diameter; n = 10), in the left atrium (n = 2), and in inferior vena cava (n = 1). All implantations in atrial septal defects were successful, with device/defect ratio ranging from 1.3 to 1.9 (median, 1.3), with no residual leak by angiography or echocardiography in seven (3/10 had/= small immediate leaks). The STARFlex system was effective in closing created atrial septal defects using a 10 Fr delivery sheath and low device/defect sizing ratios, comparing favorably with the standard CardioSEAL. Cathet. Cardiovasc. Intervent. 49:225-233, 2000.

When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.

The transposition of the great arteries [S,D,L] complex is delineated for the first time from the anatomic, diagnostic, and surgical standpoints in this study of 26 cases: 16 surgical and 10 postmortem. Transposition of the great arteries with situs solitus of the viscera and atria (S), D-loop ventricles (D), and L-transposition (L) was characterized by six additional interrelated anomalies that largely determined surgical management: (1) ventricular septal defect, usually conoventricular, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronary ostium resulting in "single" right coronary artery in 23%. Complete surgical repair was done in 81% of the surgical patients with a 12.5% hospital mortality rate and no late deaths. When there was no pulmonary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age. However, when pulmonary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the REV procedure (réparation a l'etage ventriculaire). When complex intracardiac anatomy precluded biventricular repair, a palliative procedure was performed in 19% without mortality. Hence, this experience indicates that surgical management of patients with the transposition of the great arteries [S,D,L] complex is feasible.

Information on surgical management and outcome in patients who develop symptomatic right ventricular failure after prior Mustard or Senning operations is limited.From March 1987 to March 1991, 10 patients 3.6-23.5 years old (median, 7.0 years) with transposition of the great arteries and prior Mustard (six patients) or Senning (four patients) repairs (performed at ages 2 months to 5 years; median, 6 months) underwent surgical intervention for symptomatic right ventricular failure. In five of 10 patients, anatomic correction with either an arterial switch operation (three patients) or a pulmonary artery-to-aorta anastomosis and right ventricle-to-pulmonary artery conduit (two patients) was performed. Before anatomic correction in these five patients, four of five patients had a pulmonary artery band to prepare the left ventricle. The interval between preparation and correction ranged from 8 days to 12 months (median, 2 months). One patient died after an arterial switch operation. In the remaining five patients, coexisting left ventricular dysfunction precluded anatomic correction; all five patients survived cardiac transplantation. Survival for the entire group of 10 patients is 90%, and the median postoperative hospital stay was 17 days. During follow-up (12-62 months; median, 27 months), there were no deaths. Neoaortic insufficiency after anatomic correction was common (mild in one patient, moderate in two patients, and severe in one patient who required aortic valve replacement 4 months after surgery). In the transplantation group, one patient developed lymphoma 3 months after transplantation but is currently in remission after reduction of immunosuppression.In patients who develop late right ventricular failure after Mustard or Senning repair, surgical intervention with either anatomic correction or cardiac transplantation can be done with acceptable morbidity and low mortality. Neoaortic valve insufficiency demands close follow-up after anatomic correction.

BACKGROUND In adults, acquired pressure-overload left ventricular hypertrophy can result in myocardial ischemia, which may be due in part to insufficient capillary growth during development of hypertrophy. The coronary microvascular response to congenital pressure-overload hypertrophy in children has not been previously characterized. METHODS AND RESULTS Average capillary density and heterogeneity of capillary spacing were measured in 63 postmortem human hearts with left ventricular hypertrophy and control hearts without heart disease. Pathology specimens were chosen that had left ventricular hypertrophy caused by 1) congenital isolated aortic valve stenosis in infants less than 1 year old at death, children 9-14 years old, and adults 15-30 years old; 2) congenital isolated coarctation of the aorta in adults 15-39 years old; and 3) acquired aortic stenosis in adults 51-86 years old. Major findings of the study were: 1) Human left ventricular capillary density and heterogeneity of capillary spacing are similar to other mammalian species. 2) Capillary density is higher in infants (3,315 +/- 85 capillaries per square millimeter), decreases with increasing heart weight during normal growth in early childhood (children, 2,388 +/- 75 capillaries per square millimeter, p less than 0.05), and thereafter remains relatively constant. 3) Capillary density with left ventricular hypertrophy is dependent on the age of onset. Congenital aortic stenosis and coarctation are characterized by an increase in capillary supply proportional to myocyte volume, maintaining capillary density similar to control hearts. Adults with acquired aortic stenosis have decreased capillary density (1,671 +/- 66 capillaries per square millimeter, p less than 0.01 versus control). CONCLUSIONS Pressure-overload left ventricular hypertrophy in children demonstrates proportional capillary angiogenesis, whereas in adults, hypertrophy appears to be associated with failure of compensatory angiogenesis.

1. The arterial switch procedure is now the operation of choice for typical D-transposition of the great arteries at The Children's Hospital, Boston, USA, the operative mortality rate since 1985 being 3%. 2. There are many anatomic considerations suggesting the morphologically left ventricle (LV) may well be a better systemic pump than the morphologically right ventricle (RV) which, if true, would favor the arterial switch procedure as opposed to an atrial switch operation: (1) The LV consists almost entirely of the sinus or pumping portion, and has only a minimal distal infundibular (conal) component. The RV, by contrast, has a relatively much larger infundibular component, the primary function of which is to prevent regurgitation rather than to pump. (2) Phylogenetically, the LV is the ancient "professional" pump. By contrast, the RV is a comparatively recent modification of the bulbus cordis. (3) The LV is a two-coronary ventricle, whereas the RV is a one-coronary ventricle. (4) The LV has relatively much more compact myocardium (stratum compactum) than does the RV. (5) The mitral valve leaflets are better designed to occlude a circular systemic atrioventricular orifice than are the tricuspid valve leaflets. (6) The papillary muscles of the LV are large, paired, well balanced, and both arise from the same ventricular wall--the LV free wall. By contrast, the papillary muscles of the RV are comparatively small, numerous, unbalanced, and arise from both ventricular septal and free walls. Hence, dilatation of the LV does not pull the LV papillary muscles apart, whereas dilatation of the RV does pull the RV muscles apart, favoring the development or exacerbation of tricuspid regurgitation. (7) The LV has two conduction system radiations, whereas the RV has only one. 3. The current anatomic contraindications to the arterial switch operation in typical D-TGA include the following: (1) an unprepared LV; (2) an aortic intramural left coronary artery arising from the right coronary sinus of Valsalva; (3) pulmonary outflow tract stenosis (with small annulus and subvalvar obstruction) or atresia; (4) aortic outflow tract stenosis (with small annulus and subvalvar obstruction) with tubular hypoplasia of the aortic arch and preductal coarctation; (5) tricuspid or mitral atresia; (6) marked underdevelopment or absence of either the RV sinus or the LV sinus; and; (7) 2 major anomaly of the systemic and/or pulmonary veins, as in the heterotaxy syndrome with asplenia.(ABSTRACT TRUNCATED AT 400 WORDS)

Two cases of nonimmune hydrops fetalis and fetal death associated with cardiac rhabdomyoma are reported. Case 1 presented with fetal supraventricular tachycardia, and cardiac rhabdomyoma was accurately diagnosed by fetal echocardiography. Autopsy revealed multiple rhabdomyomata involving the right atrial free wall, the sinoatrial node, and the left ventricle. The left circumflex coronary artery was extrinsically compressed by adjacent tumor tissue, causing left ventricular myocardial infarction. Case 2 had a unique, pedunculated, ball-like rhabdomyoma that almost totally occluded the mitral orifice. The causes of fetal death in patients with cardiac rhabdomyoma are analyzed and the possibility of fetal surgical management is proposed.

A summary and brief illustration of the morphologic-anatomic method of diagnosis of congenital heart disease is presented. The principles of scientific neologizing are considered and exemplified. Scientific freedom of speech and expression is commented upon. It is suggested that unnecessary renaming of numerous cardiac anatomic structures and many forms of congenital heart disease be discontinued, and that terminology be de-emphasized.

A technique for extensive resection of the infundibular septum through the superior transarterial approach is proposed. Infundibular septal resection facilitates intraventricular rerouting for anatomic correction of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle. This technique also may be used to enlarge progressively obstructive but physiologically advantageous ventricular septal defects, for example, with tricuspid atresia. Through the superior transaortic or transpulmonary approach, the landmarks of the infundibular septum are the intercoronary commissure of the aortic valve and the septal commissure of the pulmonary valve, both of which are directly above the middle of the infundibular septum. With this exposure, the infundibular septum may then be resected easily and relatively completely and a large opening created immediately beneath the semilunar valves. The feasibility of infundibular septal resection by the superior approach was assessed post mortem in 25 cases of transposition of the great arteries with ventricular septal defect and in 20 autopsied cases of double-outlet right ventricle. This technique was subsequently applied successfully to enlarge the obstructive ventricular septal defect of a 2-year, 11-month-old girl with D-transposition of the great arteries. The advantages of the proposed technique include technical ease, relative completeness of infundibular septal resection, and the facilitation of intraventricular repair of transposition of the great arteries with ventricular septal defect and double-outlet right ventricle.

Total anomalous pulmonary venous connection (TAPVC) is failure of development of the common pulmonary vein, with consequent ersistence and enlargement of embryonic collaterals between the lungs and the systemic veins. In the great majority (91 of 93 autopsied cases, 97 per cent, no remnant of the common pulmonary vein was found, supporting the concept of agenesis or involution of the common pulmonary vein. Occasionally, a cordlike strand was found in the location of the common pulmonary vein (in three of 93 cases, 3 per cent), supporting the concept of atresia of the common pulmonry vein. The order of frequency of the various types of TAPVC was: left innominate vein ("snowman"), 26 perent cent; subdiaphragmatic, 24 per cent; coronary sinus, 18 per cent; right superior vena cava, 15 per cent; right atrium, 8 percent; mixed, 5 per cent; azygos, 2 per cent; and left supeior vena cava, 2 per cent...

An essentially single focus of left ventricular chordal insertion was found in 23 of 164 autopsied cases of common atrioventricular canal (CAVC) with a normally formed spleen (14%). Suture closure of the cleft of the mitral valve in such cases results in the surgical creation of parachute mitral valve, often with fatal iatrogenic mitral stenosis. In such patients, the cleft of the mitral valve is its main orifice and must not be sutured closed. There are four anatomic types of potentially parachute mitral valve in CAVC: (1) with one papillary muscle group and one mitral orifice (type 1A), in 10 cases (43%); (2) with one papillary muscle group and two mitral orifices (type 1B), in one case (4%); (3) with two papillary muscle groups and one mitral orifice (type 2A), in five cases (22%); and (4) with two papillary muscle groups and two mitral orifices (type 2B), in seven cases (30%). Parachute mitral valve exists only when the AVC is divided, either naturally or surgically. The essence of parachute mitral valve is an essentially single focus of chordal insertion. One or both left ventricular papillary muscle groups may be present. The presence of only one focus of left ventricular chordal insertion contraindicates cleft closure.

Electrophysiologic delineation of the atrioventricular conduction system at surgery is described in two patients with corrected transpostion of the great arteries in situs inversus. Intra-atrial electrograms were recorded in one patient from sites immediately adjacent to the coronary sinus located in the left-sided right atrium. The intraventricular portion of the atrioventricular conduction system was identified in both patients along the posterior and inferior margin of the ventricular septal defect, in contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. In contrast to the superior and anterior location found in corrected transposition of the great arteries in situs solitus. The course of the conduction system in the hearts of these two patients and a possible relationship to the cardiac loop and dual origin of the atrioventricular node is discussed. These cases illustrate the usefulness of segmental diagnosis of congenital heart disease and of electrophysiologic identification of the specialized atrioventricular conduction system at surgery.

Salient clinical, hemodynamic, angiocardiographic, and pathologic findings are presented in 100 patients with truncus arteriosus communis, 79 of whom were studied at autopsy. In this study of typical truncus, all had a ventricular septal defect (type A). Truncus with a partially formed aorticopulmonary septum (type A1) was much the commonest form (50%). Cases with no remnant of aorticopulmonary septum (type A2) were second in frequency (21%). The distinction between types A1 and A2 could not be made with certainty in 9%, because these types merge into one another. Cases with absence of either pulmonary artery branch (type A3) were the least frequent form (8%). Truncus with interruption, atresia, preductal coarctation, or severe hypoplasia of the aortic arch (type A4) constituted 12%. The diagnosis of truncus is primarily angiocardiographic. The plane of the truncal valve in the lateral projection is distinctive. It tilts anteriorly, facing the patient's toes, which can be of assistance in differential diagnosis. The angiocardiographic features of type A4, although unfamiliar, are pathognomonic. Since the median age at death was only 5 weeks, and in view of the difficulties associated with pulmonary artery banding, our goal should be the surgical correction of truncus during the first and second months of life.

Recent experience with the spring-loaded patent ductus arteriosus (PDA) occluder has demonstrated several advantages of this device in the closure of intracardiac defects compared with previously described techniques. Pathologic and animal model studies were performed to identify which atrial septal defects (ASDs) might be suitable for device closure and to test a new septal closure double umbrella. Fifty specimens from the Cardiac Registry with unrepaired ASD secunda (2 degrees) were analyzed. Mean ASD size was 8 x 10 mm (range, 3 x 4 to 30 x 30 mm); 80% (n = 40) of ASDs were judged closable with the new device. ASD closure was attempted in four lambs with the Rashkind (hooked single umbrella) ASD occluder. One of four umbrellas was correctly positioned; no ASDs were closed. A new double-hinged ("clamshell") umbrella device was designed: eight ASD closures were attempted with this device (defects ranged from 8 to 20 mm in diameter). Six of eight umbrellas were correctly positioned; four of four animals observed more than 1 day appeared to have complete closure on postmortem examination with endothelialization of the device. We conclude that 1) most ASD 2 degrees are far enough from vital structures to permit closure, 2) initial placement of hooked umbrellas is often incorrect and cannot be altered, 3) clamshell double umbrellas were successfully placed in six of eight attempts, and 4) endothelialization of closed ASDs appears complete within weeks of implantation. These preliminary studies appear promising and support testing the clamshell ASD device in clinical trials.

Two autopsy-proved cases are presented of a rare form of congenital heart disease, anatomically corrected malposition (S, D, L). Anatomically corrected malposition means that despite the abnormal relationship between the great arteries, the aorta arises nonetheless above the anatomically left ventricle and the pulmonary artery originates above the anatomically right ventricle. (S, D. L) briefly indicates the segmental set or combination: situs solitus of viscera and atria (S), ventricular D-loop (D), AND L-MALPOSITION OF THE GREAT ARTERIES (L). These are the first cases of anatomically corrected malposition (ACM) in whom the presence of a subaortic muscular conus only has been documented pathologically. This established that ACM can occur with a subaortic conus, as well as with a bilateral conus. From the diagnostic standpoint, the distributions or epicardial courses of the coronary arteries are recommended as a helpful approach to ventricular identification. This diagnostic method has widespread angiocardiographic and surgical applications. Symbolic terminology, exemplified by anatomically corrected malposition (S, D. L) is brief and accurate. This approach may conveniently be applied to many other forms of comples congenital heart disease, and it considerably facilitates computer data processing.

The clinical, hemodynamic, angiocardiographic, and postmortem findings of a previously unreported type of subaortic stenosis are presented in two patients who also had straddling tricuspid valve and transposition of the great arteries. The subaortic stenosis became apparent after banding of the main pulmonary artery and was due to hypertrophy of a probably abnormally positioned moderator band resulting in a double-chambered right ventricle. Fibrous tissue accumulation at the stenotic os infundibuli also contributed to the subaortic obstruction. Both patients had situs solitus of the atria. Patient 1 had ventricular inversion (L-loop ventricles) and atresia of the right-sided mitral valve. Patient 2 had normally positioned ventricles (D-loop) and two atrioventricular valves. The presence of a large left ventricle and a small right ventricle in the angiocardiogram led to the erroneous diagnosis of a single left ventricle with an infundibular outlet chamber in both patients. Consequently, the subaortic obstruction was thought preoperatively to be at the site of a restrictive bulboventricular foramen. Patient 1 died 36 hours after placement of a valved conduit from the left ventricle to the descending aorta. Patient 2 was operated on successfully and the surgical procedures performed are described.

1. Surgical repair of origin of a pulmonary artery branch from the ascending aorta should be performed as early as possible in order to prevent death from congestive heart failure or the development of irreversible pulmonary vascular obstructive disease. 2. Deep hypothermic circulatory arrest greatly facilitates surgical repair of this lesion in infancy, as is illustrated by the three cases reported herein. 3. Direct anastomosis of the ectopic pulmonary artery branch to the main pulmonary artery is the surgical technique of choice. 4. Origin of the RPA or the LPA from the ascending aorta results from origin of the RPA or LPA from the aortic sac, instead of from the confluent sixth arches. Typically, the RPA has failed to migrate leftward because of abnormal development of the wall of the aortic sac, abnormal development of the sixth arches, or both. 5. Origin of the RPA or the LPA from the ascending aorta should be distinguished from origin of the "RPA" or of the "LPA" from the innominate artery or from the aortic arch via a PDA or a collateral artery.