Gradient reduction, aortic valve regurgitation and prolapse after balloon aortic valvuloplasty in 32 consecutive patients with congenital aortic stenosis.

From 1986 to 1988, balloon aortic valvuloplasty was performed in 32 patients with congenital valvular aortic stenosis. The patients ranged in age from 2 days to 28 years (mean +/- SD 8.3 +/- 5.9). One balloon was used in 17 patients and two balloons were used in 15 patients. Immediately after valvuloplasty, peak systolic pressure gradient across the aortic valve decreased significantly from 77 +/- 27 to 23 +/- 16 mm Hg (p less than 0.01), a 70% reduction in gradient. At early follow-up study (4.1 +/- 3.3 months after valvuloplasty), there was a 48 +/- 20.5% reduction in gradient compared with that before valvuloplasty, and at late follow-up evaluation (19.2 +/- 5.6 months), a reduction in gradient of 40 +/- 29% persisted. Echocardiography showed evidence of significantly increased aortic regurgitation in 10 patients (31%) and aortic valve prolapse in 7 patients (22%). There was no correlation between the balloon/anulus ratio and the subsequent development of aortic regurgitation or prolapse. In fact, no patient who showed a significant increase in aortic regurgitation had had a balloon/anulus ratio greater than 100%. It is concluded that balloon aortic valvuloplasty effectively reduces peak systolic pressure gradient across the aortic valve in patients with congenital aortic stenosis. However, subsequent aortic regurgitation and prolapse occur in a significant number of patients, even if appropriate technique and a balloon size no greater than that of the aortic anulus are used.

Maturational changes in left and right ventricular electromechanical intervals in the newborn lamb.

Electromechanical intervals of right and left ventricles were examined in newborn lambs (9 hours to 42 days). The electromechanical interval of the left ventricle did not change with maturation, while that of the right ventricle became progressively longer, resulting in an increasing ventricular mechanical asynchrony. We postulate that the pattern of developing mechanical asynchrony described may occur secondary to postnatal changes in ventricular morphology.

Echocardiographic features of total anomalous pulmonary venous connection to the coronary sinus.

Six infants with anomalous connection of the pulmonary veins to the coronary sinus were studied with single crystal echocardiography. The posterior left atrial wall echo was identified by its continuity with the left ventricular posterior wall echo during a sweep from the left atrium to the left ventricle. In five of the six infants an echo-free space measuring 7 to 9 mm was identified behind the posterior left atrial wall; this space was thought to represent the common pulmonary vein. In four of these five, and also in the remaining infant, a highly mobile linear echo with a double wave form per cardiac cycle was recorded behind the posterior aortic wall and anterior mitral leaflet echoes. Echocardiographic contrast studies after the injection of saline solution into the coronary sinus disclosed that this additional echo was produced by the anterior wall of the coronary sinus. The anatomic connection of the pulmonary veins were established in each patient with pulmonary cineangiography. In four of the six patients all four pulmonary veins were connected to the coronary sinus by way of a common pulmonary vein; in the remaining two patients three of four pulmonary veins were connected to the coronary sinus, while the left upper lobe pulmonary vein was connected to the left innominate vein. The coronary sinus was greatly enlarged in each patient.

Familial total anomalous pulmonary venous return: a large Utah-Idaho family.

Total anomalous pulmonary venous return (TAPVR) is a rare form of cyanotic congenital heart disease which, without surgical correction, has a high mortality rate in the first year of life. It usually occurs without a family history, and is generally thought to have a low recurrence risk. However, 15 instances of familial TAPVR have been reported previously, suggesting a genetic cause in some cases. We report on a large family in which TAPVR appears to be determined multifactorially or as an autosomal dominant trait with variable expressivity and reduced penetrance.

Right atrial ball-valve thrombus: a complication of central venous alimentation in an infant. Diagnosis and successful surgical management of a case.

The following is a case report of a 6-week-old premature infant in whom a ball-valve thrombus developed after prolonged central venous alimentation. Clinical presentation included facial edema, cyanotic episodes, and apnea. No murmur was present, but the diagnosis was suspected when a calcified right atrial mass became apparent on the plain chest film. The diagnosis was confirmed by echocardiography and then venous and cardiac angiography. The calcified thrombus was removed successfully from the right atrium by use of profound hypothermia with ether anesthesia and total circulatory arrest. Subsequently, the patient made an uneventful recovery and is healthy 3 years postoperatively.

Early and late results in pulmonary atresia and intact ventricular septum.

We examined the early and late results of operations in 29 consecutive neonates with pulmonary atresia and intact ventricular septum treated from 1980 to 1988. Transventricular pulmonary valvotomy and central aorta-pulmonary artery shunting were performed in 19 of 22 infants who had a patent infundibulum. Pulmonary valvotomy alone was performed in 3 of the 22 infants with a patent infundibulum, but 2 of these required subsequent systemic-pulmonary artery shunts. Primary shunting was used to palliate 7 infants who had absent infundibular portions of the right ventricle and a very diminutive right ventricular cavity. Tricuspid valve excision and atrial septectomy were also performed in 5 of these 7 infants to decompress large fistulous communications between the right ventricule and coronary artery. Two early deaths (2/29, 6.9%) occurred overall. Both were in infants who had a very small right ventricle. Definitive operation has been accomplished in 16 patients; 13 have had closure of residual interatrial communications and shunt ligation with no deaths, and 3 have undergone modified Fontan repair with 1 death. Actuarial survival rate for the entire group, including operative deaths, is 86% at 5 years. The technique of transventricular pulmonary valvotomy and systemic-pulmonary artery shunting offers a reliable means of palliating neonates with pulmonary atresia and intact ventricular septum and obtains good late right ventricular growth. Systemic-pulmonary shunting, tricuspid valvectomy, and atrial septectomy may offer a means of reducing or obliterating right ventricular-coronary artery fistulas.

An unusual cause of severe cyanosis in infancy.

An infant presented with cyanosis due to a diaphragmatic Morgagni hernia compromising right ventricular diastolic filling and resulting in right-to-left atrial-level shunting as demonstrated by contrast echocardiography. There was complete resolution of cyanosis after repair of the hernia.

Ligation of the patent ductus arteriousus in the newborn intensive care unit.

During a two-year period, 21 premature infants (weight at birth, 680 to 2,340 gm) had operative closure of patent ductus arteriosus (PDA). The first 6 infants had ligation performed in the operating room (OR); the subsequent 15 had ligation in the Newborn Intensive Care Unit. There were no immediate postoperative deaths. Two infants died from problems presnet preoperatively within 30 days postoperatively. There were no infections. Technique in the unit utilizes an open warmer with local anesthesia and a paralyzing agent. By eliminating transportation to the OR one avoids problems with thermoregulation, loss of lines, malfunction of monitors, poorly controlled ventilation, and fluid overload. Additional advantages to ligation in the unit are that the infant is already monitored, intubated, and on a respirator, and that venous and usually umbilical arterial lines are in place. At the conclusion of operation, management is returned to the neonatologists for optimal continuity of care.

Absorbable polydioxanone suture and results in total anomalous pulmonary venous connection.

BACKGROUND: Despite theoretical advantages of absorbable suture in the growing vascular anastomosis, there has not been a documented advantage over nonabsorbable suture in preventing late anastomotic stenosis in total anomalous pulmonary venous connection (TAPVC). METHODS: We reviewed our experience from 1982 to 1994 with 65 hospital survivors of total TAPVC repair to examine the influence of suture type on survival and incidence of late pulmonary venous obstruction. From 1982 until 1988, we used continuous nonabsorbable polypropylene suture for the pulmonary venous-left atrial anastomosis in supracardiac, infracardiac, and mixed types of TAPVC: In 1989, we adopted a running absorbable polydioxanone suture technique. Cardiac catheterization and echocardiography were used to evaluate late pulmonary venous obstruction. RESULTS: Late pulmonary venous obstruction occurred in 17% (4/23) of survivors after repair with polypropylene suture compared with 3.2% (1/32) after repair with polydioxanone suture (p < 0.05). There were no instances of late pulmonary venous obstruction in the intracardiac TAPVC group (0/10). All late pulmonary venous obstructions occurred within 16 months after operation. The actuarial 3-year and 5-year freedom from late pulmonary venous obstruction was 100% for intracardiac TAPVC, 96% for the polydioxanone group, and 81% for the polypropylene group. Five patients died late (5/65, 7.7%), 3 in the polypropylene suture group (3/23, 13%) and 2 in the polydioxanone group (2/32, 6%). CONCLUSIONS: Continuous absorbable polydioxanone suture for the repair of TAPVC results in a low incidence of late pulmonary venous obstruction and death and appears to offer advantages over a continuous nonabsorbable suture. A continuous nonabsorbable suture may limit growth of a vascular anastomosis, particularly one involving a "low-pressure" anastomosis such as in the repair of TAPVC:

Acute rheumatic fever in the 1980s.

Acute rheumatic fever (ARF) is an inflammatory sequela which occurs in 1-3% of children afflicted with group A beta-hemolytic streptococcal pharyngitis (strep throat). The major manifestations are carditis, migratory polyarthritis and chorea. ARF recurs with repeated strep throats and frequently leads to rheumatic heart disease (RHD), usually mitral and aortic regurgitation and mitral stenosis. ARF likely results from an abnormal host immune response with a host-antibody/streptococcal antigen production in pharyngeal tissue and subsequent cross-reaction of host antibodies with host end organs. Treatment includes eradication of the streptococcus, use of high doses of salicylates and adrenal corticosteroids, and prolonged bed rest with gradual ambulation after clinical and laboratory signs of the disease are gone. While the incidence and mortality of ARF and RHD have decreased drastically in the affluent industrialized countries of Europe, North America, and in Japan, the disease is a major health problem in the less affluent, 'developing' countries of Latin America, the Middle East, Africa, India and Southeast Asia. The major risk factors for ARF are believed to be genetic or familial, inadequate medical care and crowded conditions. The last two factors are socioeconomic but may also be influenced by ethnic cultural behavior. Genetic propensity for ARF is supported by recent evidence of a specific DR-HLA marker in the majority of people with ARF or RHD. Finally, while ARF appears to be vanishing in most areas of the country, it is still prevalent in some affluent populations and in some disadvantaged minorities.

Pre- and postoperative exercise testing of the child with coarctation of the aorta.

Exercise studies in patients with coarctation of the aorta (CoA) are reviewed. A brief survey of the natural history of unoperated CoA, the pioneers who reported the first operative repair, and comments on blood pressure measurements are provided. Preoperative and postoperative (postop) exercise studies of patients with CoA are also reviewed. The various etiological factors which may contribute to hypertension found in some post-op CoA patients are discussed.

Ventricular epicardial activation sequence in the lamb.

The ventricular epicardial activation sequence (VEAS) in 15 anesthetized lambs (near term fetus to 3.5 months of age) was determined using 40 simultaneously recorded bipolar electrograms for each animal. Isochrone maps were drawn by hand using relative activation times determined from the maximum first derivative of multiplexed signals recorded and analyzed by computer from single cardiac cycles. VEAS was similar for all ages studied. Earliest left ventricular (LV) activity appeared on the caudal dorsal and/or ventral free wall. Initial right ventricular (RV) activation appeared on the ventral anterior and/or lateral surface either simultaneously with or slightly later (2.5-3.5 msec) than initial LV activation. Excitation then proceeded circumferentially and in an apicobasilar direction and terminated on the right ventricular outflow tract.(RVO). All LV and RV epicardium except RVO activated within 7.5-12.5 msec. RVO required more than 1n.5 msec to activate in 12 of 15 animals. Total duration of RVO activation never exceeded 22.5 msec in any animal. Neither the duration nor the pattern of activation of LV or RV epicardium, including RVO, changed in a consistent fashion with age. These findings are similar to the known VEAS for adult ruminants. The data indicate that the VEAS assumes the adult pattern as of late gestation and suggest that changing right and left ventricular electromechanical events do not contribute to and probably are not affected by the process of ventricular epicardial activation. Speculation Although maturational changes in right and left ventricular electromechanical events do not appear to be related to the sequence of ventricular epicardial activation, such changes may be related to the distrubution of corresponding epicardial and intramural isopotentials.