Subpulmonary Ventricular Septal Defect Research Articles

Surgical results in patients with double outlet right ventricle: a 20-year experience

Background. The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. Methods. Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle. Results. Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively ( p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 ± 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively ( p = 0.11). Conclusions. Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.

Transposition Complexes With Systemic Obstruction

The arterial switch operation is currently the procedure of choice for transposition of the great arteries and double-outlet right ventricle with subpulmonary ventricular septal defect. While the results of surgical repair have improved tremendously in recent years, the presence of associated lesions continues to make this a surgically challenging malformation. The association of these so-called transposition complexes with systemic obstruction has recently received increased attention. Systemic obstruction may occur at the subaortic level, in the aortic arch, or at both levels. Valvar aortic stenosis or atresia is extremely rare. Resection of hypertrophied muscle bundles with or without pericardial patch augmentation is frequently enough to deal with obstruction at the subaortic level, which becomes the subpulmonary area following arterial switch operation. Aortic arch obstruction associated with intracardiac defects has traditionally been addressed with a staged approach, dealing first with the arch obstruction followed later by intracardiac repair. The results with this approach have been disappointing. At the Montréal Children's Hospital, we have obtained superior results using a single-stage approach. Therefore, we have advocated the use of pulmonary homograft patch aortoplasty for aortic arch reconstruction at the time of intracardiac repair to completely remove any anatomic afterload. Since 1989, in 22 consecutive patients undergoing single-stage anatomic repair of transposition complexes associated with aortic arch obstruction, we have had no early deaths, one late death of a noncardiac cause, and one recoarctation requiring balloon dilatation. In the last 2 years, we have been able to perform all our aortic arch reconstructions avoiding the use of circulatory arrest.

Surgical outcome of double-outlet right ventricle with subpulmonary VSD

Background. Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration. Methods. Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 ± 18 months. Results. An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%). Conclusions. The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.

Surgical management of the straddling mitral valve in the biventricular heart

Background. The straddling mitral valve in the biventricular heart is a rare condition that may complicate biventricular repair.Methods. Treatment and outcomes in 5 consecutive patients who underwent primary repair between 1992 and 1997 were reviewed. Their ages at repair ranged from 2 months to 8 years. Three patients had a double-outlet right ventricle with a subaortic (n = 2) or subpulmonary (n = 1) ventricular septal defect. Two patients had transposition of the great arteries {S,D,D}, a ventricular septal defect, and left ventricular outflow tract obstruction. The attachments of the papillary muscles of the straddling mitral valves were located on the right ventricular aspect of the ventricular septum. Four patients underwent baffle partitioning of the ventricular cavity. The baffle suture line was used to secure the chordae tendineae crossing the ventricular septal defect, or was intentionally omitted at the papillary muscle. The right ventricular outflow tract was reconstructed with patch augmentation, an extracardiac conduit, or an arterial switch operation. One patient with transposition who had a giant papillary muscle to the straddling mitral valve associated with abnormal insertion of the tricuspid valve on the conal septum underwent univentricular repair.Results. There were no early or late postoperative deaths. There was no mitral valve dysfunction, left ventricular outflow tract obstruction, or heart block in the 4 patients who underwent biventricular repair.Conclusions. Although there are several exceptional situations in which ventricular partitioning may result in early and late complications, a straddling mitral valve does not preclude biventricular repair.

Ventricular outflow tracts after Kawashima intraventricular rerouting for double outlet right ventricle with subpulmonary ventricular septal defect.

To determine whether or not the ventricular outflow tracts can be efficiently constructed in patients with double outlet right ventricle with subpulmonary ventricular septal defect by the Kawashima intraventricular rerouting in which the morphologically right ventricular outlet is divided into two, one for the systemic and the other for the pulmonary circulations. The intraventricular rerouting procedure was carried out in nine patients with this particular malformation. Age at repair ranged from 35 days to 3 years old. The distance between the attachments of the tricuspid and the pulmonary valves was 10 mm or greater in all except one patient in whom the measured value was 3 mm. Resecting subaortic musculature appropriately, a tailored patch, either oval-shaped (in seven) or heart-shaped (in two), was placed to construct an unobstructed channel for the left ventricular outflow tract with its diameter greater than that of the anticipated normal aortic orifice at the time of repair. For an unobstructed channel to the pulmonary arteries, enlargement of the right ventricular outflow tract was carried out using a patch in six. All patients survived the operative procedure. On postoperative catheterization, mean pulmonary arterial pressure was 15 +/- 8 mmHg, and cardiac index was calculated as 3.3 +/- 0.6 l/min per m2. It proved that the constructed left ventricular outflow tract can become larger in the longer term. Pressure gradient across the left ventricular outflow tract was greater than 20 mmHg in two patients in the intermediate term. One of these two underwent reoperation for the obstruction 10 years after the initial repair. It was suspected that use of a heart-shaped internal conduit, which seems to result from inadequate conal resection, was one of the possible causes of such obstruction in the longer term. Pressure gradient of 47 mmHg was seen across the right ventricular outflow tract in one patient, although this patient has undergone no reoperation. Enlargement of the right ventricular outflow tract could minimize postoperative obstruction for the pulmonary pathway. The intraventricular rerouting remains one of the attractive surgical options for repair in this particular setting, in terms of successful construction of the ventricular outflow tracts.

Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies

OBJECTIVESThe purpose of the study was to determine the accuracy of the prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies.BACKGROUNDThe accuracy of prenatal echocardiographic diagnoses of cardiac lesions has been reported, but no previous reports specifically address fetal conotruncal anomalies.METHODSMedical records of 61 fetuses, in which a fetal diagnosis of a conotruncal anomaly was made, were reviewed. Disease entities included were tetralogy of Fallot (TOF), double outlet right ventricle (DORV), transposition of the great arteries (TGA), and truncus arteriosus (TA).RESULTSFetal diagnosis was established at a median of 24.5 weeks’ gestation. Termination of pregnancy was chosen in 31% (19/61) of cases. Postnatal assessment of the diagnosis was not obtained in 12 cases. Excluding two sets of conjoined twins, accurate prenatal diagnosis including definition of the great artery orientation was achieved in 36 of 47 cases (77%). Seven of 17 fetuses with DORV anatomy, of which 6 were thought to have a subpulmonary ventricular septal defect (VSD), had incorrect prenatal assessment of the great artery relationships. One fetus thought to show features of TA had aortic atresia with VSD and normal-sized left ventricle. Of the 42 pregnancies that continued, 15 had major extracardiac malformations and/or chromosomal abnormalities of which one died in utero with trisomy-13 and TA. A further nine died within the neonatal period. Among the 27 fetuses without a documented chromosomal or major extracardiac anomaly, 13 (48%) died. Overall, the survival rate beyond 28 days of life was 52% (22/42). In contrast, 75% (6/8) of fetuses with TOF, excluding the absent pulmonary valve syndrome, survived.CONCLUSIONSConotruncal anomalies can be diagnosed by prenatal echocardiography with a high degree of accuracy. Defining the exact spatial relationship of the great arteries is problematic in some fetuses. The overall prognosis for fetuses with a conotruncal anomaly is poor, with the exception of uncomplicated TOF.

Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD.

An arterial switch operation is considered a good alternative for the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) when intraventricular rerouting is not feasible. The clinical results of an arterial switch operation with ventricular septal defect closure for this anomaly were studied. Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch operation with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to define double-outlet right ventricle. Arch anomalies were associated in nine cases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of the ventricular outflow tract. The relationship of the great arteries was mostly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary artery in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side by side relation). The Lecompte maneuver was used to reconstruct the pulmonary artery in all but five cases with a side by side relationship of the great arteries. There was one operative death (3.7%) and three late deaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricular outflow tract obstruction including peripheral pulmonary stenosis developed in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. Although double-outlet right ventricle with subpulmonary VSD has complex features, including an aortic arch obstruction and coronary artery anomalies, an optimal definitive surgical repair using an arterial switch operation can be performed safely with a thorough understanding of this variable anomaly. The prevention of right ventricular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity.

Anomalous right coronary artery from the pulmonary artery in Taussig-Bing anomaly

The presence of associated anomalies in patients with double-outlet right ventricle can significantly alter surgical intervention. Preoperative delineation of these anomalies can facilitate surgical planning and improve outcome. We describe a case in which the right coronary artery and anterior descending coronary artery arose from the pulmonary artery in a patient with double-outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly). Recognition of this important anomaly prevented significant intraoperative myocardial damage by altering techniques of cardioplegia administration for myocardial preservation.

Single-Stage Arterial Switch With Aortic Arch Enlargement for Transposition Complexes With Aortic Arch Obstruction

Background. Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results. Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch. Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view. Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.

Aortic Valve Prolapse and Aortic Regurgitation Associated With Subpulmonic Ventricular Septal Defect

The natural development of aortic valve deformity was studied in 315 patients with subpulmonic ventricular septal defect. The patients with early development of aortic regurgitation had a pulmonary-to-systemic flow ratio of > 1.5, normal right ventricular pressure, and severe aortic regurgitation.

Taussig-Bing anomaly and arterial switch: aortic arch obstruction does not influence outcome.

Aortic arch obstruction is a commonly associated problem in the Taussig-Bing anomaly. Between 1983 and 1995, 28 consecutive patients with Taussig-Bing anomaly underwent arterial switch operation with baffling of the left ventricle to neoaorta. Group A: 11/28 had associated aortic arch obstruction. Group B: 17/28 had isolated Taussig-Bing anomaly. We assessed whether the coexistence of subpulmonary ventricular septal defect and aortic arch obstruction pose an incremental risk factor. Group A: Mean age and weight were 1.4+/-1.3 months and 3.5+/-0.4 kg. The aortic arch obstruction included: hypoplasia (5/11), interruption (4/11) and discrete coarctation (2/11). Seven patients had a one-stage correction, and four had initial arch repair followed by arterial switch operation. There were no hospital deaths (CL 0-28%). Over a follow-up of 638 patient-months (mean 64+/-39), there have been no late deaths, and all patients are in New York Heart Association class 1. There have been three cases of recurrent aortic arch obstruction (two requiring reoperation, and one requiring balloon dilation). One patient has been reoperated upon for right ventricular outflow tract obstruction. The actuarial survival and freedom from reoperation rates at 6 years were 100% (CL = 66-100%) and 72.9% (CL=38-92%) respectively. Group B: Mean age and weight were 5.9+/-8.4 months and 5+/-2.1 kg. All patients had a one-stage operation. There were two early deaths (11.8%, CL = 1-36%) and one late death over a follow-up of 678 patient-months (mean 52+/-31). All survivors are in New York Heart Association class 1 and there have been no reoperations. The actuarial survival and freedom from reoperation rates at 6 years were 81% (CL = 56-93%) and 100% (CL = 76-100%) respectively. 1. Aortic arch obstruction has not adversely affected early or late survival (P>.05) or late functional class. 2. Patients with Taussig-Bing anomaly and aortic arch obstruction may have a higher reoperation rate than those with normal arch anatomy. 3. Taussing-Bing anomaly, with or without aortic arch obstruction, can be repaired with arterial switch operation during the neonatal period with good outcome.

Institutional experience with a protocol of early primary repair of double-outlet right ventricle

Our institution has adopted a protocol of primary repair for all patients with double-outlet right ventricle. Since May 1989, 24 consecutive neonates and infants with double-outlet right ventricle and atrioventricular concordance (median age, 4 months) underwent anatomic biventricular repair. One patient (4%) received prior pulmonary artery banding but was still repaired as a neonate at 22 days of age. Twelve patients had a subaortic ventricular septal defect (VSD), 5 patients a subpulmonary VSD, 3 patients doubly committed VSD, and 4 patients a noncommitted VSD. Sixty-nine of 72 associated lesions were repaired simultaneously. Four types of repairs were used: intraventricular rerouting in 16 patients, arterial switch operation with VSD closure into the pulmonary artery in 4 patients, Rastelli-type repair with extracardiac conduit in 3 patients, and the Damus-Kaye-Stansel repair with concomitant repair of aortic arch obstruction in 1 patient. Ventricular septal defect enlargement was necessary in 15 patients. Repair of subpulmonary stenosis and of subaortic stenosis was carried out in 13 and 4 patients, respectively. Three patients underwent simultaneous repair of aortic arch obstruction with no mortality. Two of the patients with noncommitted VSD had simultaneous repair of complete atrioventricular canal and repair of severe pulmonary venous obstruction. The perioperative mortality was 8% (2 patients, and there was one late death (4%). Two patients (9%) underwent early successful reoperations (5 and 8 weeks postoperatively). The two reoperations were for residual VSD (1 patient) and severe mitral regurgitation (1 patient). All 21 survivors are alive at a mean follow-up of 40 months (range, 7 months to 6 years). The estimated 5-year actuarial survival is 88%, with no deaths after 2 months postoperatively. Ninety-five percent of long-term survivors have no restriction of physical activities because of cardiac status and are receiving no cardiac medications. An institutional protocol of early anatomic biventricular repair of double-outlet right ventricle in infants and neonates achieves excellent survival, making palliative operations unnecessary. Associated lesions should be repaired simultaneously. The complexity of these malformations requires a highly individualized and flexible surgical approach.

Primary Intracardiac Repair of Double Outlet Right Ventricle and Ventricular Septal Defect without Pulmonary Stenosis

From January 1991' to May 1994, 29 patients with double outlet right ventricle with ventricular septal defect, without pulmonary stenosis underwent primary intracardiac repair at the All India Institute of Medical Sciences, New Delhi. Patients were classified into 4 groups based on location of the ventricular septal defect. The ventricular septal defect was subaortic in 11, subpulmonary in 13, doubly committed subarterial in 1, and noncommitted in 4 patients. Surgical treatment consisted of intraventricular routing of the left ventricle to the aorta (17), and the left ventricle to the pulmonary artery followed by an arterial switch operation (12). There were 4 (13.9%) early deaths. Follow-up ranged from 3 months to 3 years (mean, 1.5 years). There was no late mortality. Three patients had residual ventricular septal defect, one of whom has undergone reoperation. One patient has a gradient of 25 mmHg across the left ventricular outflow tract. Double outlet right ventricle with subpulmonic ventricular septal defect was found to be a significant risk factor for early mortality (p = 0.03). The subgroup of double outlet right ventricle with subpulmonic ventricular septal defect who had a combination of single coronary artery and post arterial switch operation was particularly prone to pulmonary hypertensive crisis and hospital death (p = 0.002).

Morphological Observations on the Pathogenetic Process of Transposition of the Great Arteries Induced by Retinoic Acid in Mice

The pathogenesis of complete transposition of the great arteries (TGA) is still controversial because useful animal models have not been established. We previously reported that all-trans retinoic acid induced complete TGA at a high proportion in mice. The aim of the present study was to clarify the morphogenesis of the cardiac outflow tract in the retinoic acid-treated embryos destined to develop TGA. We first examined the morphology of TGA in mouse fetuses treated with retinoic acid to establish an animal model of TGA (experiment 1) and then examined the retinoic acid-treated embryonic hearts by means of ink injection and histology (experiment 2). All mouse fetuses and embryos showed visceroatrial situs solitus and d-ventricular loop. In experiment 1, among 45 embryos treated with retinoic acid 70 mg/kg at day 8.5 of gestation, 35 (78%) had TGA and 3 (6.7%) had a double-outlet right ventricle with a subpulmonary ventricular septal defect. In experiment 2, all hearts already exhibited d-loop at gestation day 8.5. At gestation day 9.5, conus swellings, composed of acellular cardiac jelly, where hypoplastic, and the conotruncal cavity was nonspiral or tubular. At gestation day 11.0, aberrant conus swellings located anteroposteriorly to give a straight orientation to the conotruncal cavity. At gestation day 12.0, side-by-side great arteries were transposed in that the aorta arose from the right ventricle and the pulmonary artery arose above the interventricular foramen. These results suggest that a reproducible animal model of TGA can be produced in mice by treatment with retinoic acid; that there was no loop anomaly, such as an A-loop or L-loop, in our model; and that hypoplasia of the conus swellings appears to be the primary event leading to TGA.

Coronary arterial anatomy in double-outlet right ventricle with subpulmonary VSD

We have examined 38 hearts with a double-outlet right ventricle with a subpulmonary ventricular septal defect. We divided the hearts into three groups according to the angle between the planes formed between the outlet septum and the remainder of the muscular ventricular septum; namely, at approximately right angles (15 hearts), parallel (11 hearts), and at an acute angle (12 hearts). The coronary arterial pattern corresponding to that seen in the normal heart was present in 11 hearts (73%) of the "right angle" group, in only one heart (8%) of the "acute angle" group, and in none of the "parallel" group. In contrast, the most common pattern in the setting of complete transposition was observed in none, 8%, and 91% of each group, respectively. Other diverse patterns were recognized in the hearts in the acute angle group, and the incidence of abnormal branching was significantly higher in this than in the other groups (p < 0.01). Knowledge of these anatomic variations in the course of the coronary arteries, some of which would cause problems at either definitive repair or reoperation, are essential for those seeking to achieve optimal surgical repair.

Origin of both coronary arteries from the pulmonary artery.

Origin of both coronary arteries from the pulmonary artery is generally a lethal condition from progressive ventricular failure. We report the clinical and surgical course of two infants, ages 3 and 6 months, with this anomaly. One patient had normal intracardiac anatomy with low pulmonary artery pressures (30/12 mm Hg). The second patient had a restrictive subpulmonic ventricular septal defect with a moderately elevated pulmonary artery pressure (50/13 mm Hg). Left ventricular ejection and shortening fractions were profoundly depressed in both patients. The common coronary trunk arose from the right anterior facing sinus in one patient and from the left posterior facing sinus in the other. Both patients underwent repair by direct coronary implantation to the aorta. Left ventricular function improved with shortening fractions near normal at a follow-up of 6 months for one patient and 1 year for the other. Early diagnosis and prompt repair is compatible with survival and return of normal shortening fraction.

Result of biventricular repair for double-outlet right ventricle

The choice of optimal repair for many patients with double-outlet right ventricle continues to challenge the heart surgeon. We present the results of a 10-year surgical experience with the biventricular repair for double-outlet right ventricle with situs solitus and atrioventricular concordance. Preoperative anatomic findings within this population of 73 patients are detailed. These morphologic features are correlated with type of anatomic repair and clinical outcome. Patients were classified by ventricular septal defect location. Normal coronary anatomy was found in the majority of patients with subaortic and doubly-committed ventricular septal defects. Patients with subpulmonary and noncommitted ventricular septal defects had a wide variety of coronary anatomy. Patients with subpulmonary and noncommitted ventricular septal defects also had a considerably higher prevalence of aortic arch obstruction. A tricuspid-to-pulmonary annular distance equal to or greater than the diameter of the aortic annulus was found to indicate the possibility of achieving a conventional ventricular septal defect-to-aorta intraventricular tunnel repair. Tricuspid-to-pulmonary annular distance sufficient for intraventricular tunnel repair predominates in those patients with a right posterior or right side-by-side aorta. Five types of repair were used during the study period: intraventricular tunnel repair, arterial switch with ventricular septal defect-to-pulmonary artery baffle, Rastelli-type extracardiac conduit repair, Damus-Kaye-Stansel repair, and atrial inversion with ventricular septal defect-to-pulmonary artery baffle. Overall actuarial survival estimate at 8 years is 81%. The presence of multiple ventricular septal defects and patient weight lower than the median were nearly significant risk factors for early mortality (p < 0.06). Nineteen patients (26%) required 24 reoperations. Patients with subaortic ventricular septal defects were significantly reoperation free (p < 0.05). Patients with noncommitted ventricular septal defects were at significantly higher risk for reoperation during the study period (p < 0.05). The prevalence of late right or left ventricular outflow obstruction in the nonsubaortic groups is concerning. The median age at repair in this series was 0.76 years, and there was a nonsignificant trend (p = 0.13) for early mortality in patients younger than 1 year of age. These patients tended to have other serious cardiac anomalies associated with double-outlet right ventricle that necessitated early operation. On the basis of these data, we favor early repair for double-outlet right ventricle if possible.

Double Outlet Right Ventricle Associated With Cor Triatriatum Sinistrum

Echocardiographic examination of a newborn infant showed a double outlet right ventricle with subpulmonary ventricular septal defect. The left atrium was divided by a membrane-like structure into a proximal chamber receiving pulmonary veins and a distal compartment containing left atrial appendage. To our knowledge, this is the first report of cor triatriatum sinistrum associated with double outlet right ventricle.

Ventricular septal defect creation for relief of tunnel subvalvular aortic stenosis

In infants, the management of severe tunnel subvalvular aortic stenosis associated with hypoplastic aortic annulus remains a major surgical challenge. We report a case of such lesion treated by rerouting the systemic blood flow through a created subpulmonary ventricular septal defect toward the right ventricular outflow tract and the pulmonary valve. This technique provided complete relief of the left ventricular outflow tract obstruction without the implantation of a systemic prosthetic valve.

Balloon atrial septostomy in the intensive care unit under echocardiographic control—nine years experience

SummaryBetween December 1982 and April 1991, balloon atrial septostomy was performed in the intensive care unit under echocardiographic control in 60 neonates. Of the patients, 58 had complete transposition. Two patients had double outlet right ventricle with a sub-pulmonary ventricular septal defect. Associated lesions included a patent arterial duct in 19 patients, ventricular septal defect in nine, obstruction of the left ventricular outflow tract in six, aortic coarctation in two and tricuspid atresia in one. The mean age at septostomy was four days (range 4 hours - 25 days) and the mean weight 3.19 kg (range 1.17–4.25 kg). In 39 (65%) patients, an infusion of prostaglandin was in progress prior to the septostomy and 22 (37%) were being ventilated. Standard subcostal four-chamber echocardiographic views were used to show the atrial septum and to guide the catheter used for septostomy. Venous access was obtained via the femoral vein in 43 (by percutaneous puncture in 40 and by cutdown in three) and the umbilical vein in 17. Transient atrial arrhythmias were common during the septostomy but no acute hemodynamic disturbances or deaths occurred during the procedure. The size of the atrial septal defect as measured by echocardiography after the septostomy ranged from three to 12 mm in diameter. In only one patient was this inadequate. Three (5%) patients died between two and 10 days after the septostomy, two due to necrotizing enterocolitis and one from persistent hypoxemia. One patient had a cerebral thrombosis and convulsions immediately after the septostomy but made a good neurological recovery. Corrective surgery was performed in 52 (86.6%), two (3.3%) had palliative surgery and two were considered unsuitable for total correction, of whom one has died. One patient died whilst awaiting correction. We conclude that balloon atrial septostomy using echocardiographic guidance can be safely and effectively performed in the intensive care unit.