Andreas E. Urban

Early and long.term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants

OBJECTIVE To present our 17-year experience of surgical repair of total anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and infants, with particular emphasis on the role of preoperative pulmonary venous obstruction (PVO), the management of postoperative pulmonary hypertensive crises and the long-term results. PATIENTS AND METHODS From July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300 days, had repair TAPVD in our Department. Fifty-four patients had decompensated heart failure, whereas three were moribund. The anomalous drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in 17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The repair was performed under moderate hypothermia in 15 and deep hypothermic arrest in 56 patients. In case of postoperative pulmonary hypertensive crises, artificial hyperventilation with high oxygen concentration, sedation and relaxation, inotropic support and afterload reduction with phentolamine were applied selectively. The actual follow-up is based on functional symptomatology and cardiac echo-doppler studies. RESULTS Six children (8.5%) died early: four perioperatively, two on the 2nd and 10th postoperative day, respectively. Poor preoperative condition, operation before 1987 and preoperative PVO were the main incremental risk factors for death. No death occurred among the last 38 cases. There were complications in 45 patients, in particular pulmonary hypertensive crises in 27. Two patients died late, after 80 and 118 days, respectively, of progressive intrapulmonary vein fibrosis: the second despite reoperation. The risk of late death approaches zero after 6 months. Among the 63 survivors, 56 (86%) are asymptomatic and without medication, 3 months-17 years after operation. Five have mild symptoms with ordinary physical activity. Two are lost to follow-up. CONCLUSIONS Early repair of TAPVD with aggressive management of pulmonary hypertensive crises carries low operative mortality nowadays. Preoperative PVO as a risk factor has been neutralized since 1987. Long-term results are gratifying: no late death after 6 months, no reoperation and functional good results. Progressive pulmonary vein fibrosis remains an unpredictable rare cause of death within the 1st year after surgery.

Transposition of the great arteries associated with ventricular septal defect: surgical results and long-term outcome

OBJECTIVES To identify potential risk factors influencing early and late outcome following the arterial switch operation (ASO) for transposition of the great arteries associated with ventricular septal defect including double-outlet right or left ventricle. METHODS All patients who underwent ASO in our department until August 2000 (n=105) were included in this study. There were 77 transpositions of the great arteries with ventricular septal defect, 22 Taussig-Bing hearts and six patients with double-outlet morphology. The median age at operation was 24 days. Aortic arch obstruction was present in 25 patients; in 13 of these patients, a repair with aortic arch reconstruction was done before ASO. The usual coronary artery pattern was present in 59% of the patients. In six patients, we found an intramural course of at least one coronary artery. The ventricular septal defect was closed with a patch through the right atrium (n=35), the aorta (n=25), the pulmonary artery (n=25) or the right ventricle (n=3); in 17 patients a combined approach was necessary. RESULTS There were five hospital deaths (4.7%, 95% confidence limit 2-11%). The median duration of follow-up was 72 months. Fourteen patients underwent 15 reoperations 33 months after repair (median), eight for right ventricular outflow tract obstruction or neopulmonary stenosis. Four late deaths occurred, two due to complications related to coronary artery anomalies. Statistical analysis revealed no significant risk factor whatsoever correlating with death or need for reoperation. Survival after 12 years was 91.6%, and freedom from reoperation was 82.6%. Latest follow-up data showed that 13% of patients were in NYHA class II and/or required medical treatment; 87% were in NYHA class I. CONCLUSIONS ASO associated with patch closure of ventricular septal defect can be performed early in life with a low risk of mortality (<5%), low incidence of reintervention (<15%) and promising long-term outcome.

The fate of small-diameter homografts in the pulmonary position

BACKGROUND Limited durability is expected for small homograft valves that are used to correct congenital cardiac disease. METHODS All 76 homograft valves with an internal annulus diameter ranging from 8 to 13 mm that were implanted from 1987 through 2000 in the pulmonary position were retrospectively analyzed. In each case, homograft size was normalized to the patients body surface area: z-value. For 93% (14 of 15) of the 8 to 9 mm grafts, z was less than 2. For 56% (5 of 9) of the 10 mm grafts and 98% (51 of 52) of the 11 to 13 mm allografts, z was greater than 2. Survival and freedom from complications were estimated by the Kaplan-Meier method. Homograft failure was defined as homograft replacement or late death; significant dysfunction, as homograft obstruction with an echo-Doppler gradient greater than 50 mm Hg or grade III or IV valvular insufficiency. The log-rank test was used to compare outcomes. RESULTS Seven patients died early after operation; three, late. Survival was 86.5% +/- 3.8% at 1 year and remained stable during the succeeding years. Freedom from failure for all homografts was 90.6% +/- 3.7%, 71.8% +/- 6.9%, and 61.8% +/- 9.0% at 1, 5, and 10 years, respectively. Corresponding freedom from significant dysfunction was 87.6% +/- 4.1%, 51.2% +/- 7.4%, and 10.1% +/- 8.3%. The smaller homografts (z less than 2) failed and deteriorated faster (p < 0.0001): only 32.1% +/- 13.0% were still functioning at 24 months. The larger grafts (z at least 2) retained function for the first 4 years, and 73.7% +/- 10.4% had not yet failed at 10 years. CONCLUSIONS Smaller (z less than 2) homografts (the great majority of 8 to 9 mm grafts) have to be replaced early, usually within 2 years of implantation. Larger (z at least 2) grafts (nearly all 11 to 13 mm grafts) show remarkable durability and are suitable valved conduits for establishing right ventricle to pulmonary artery continuity in neonates and young infants.

Truncus arteriosus: ten-year experience with homograft repair in neonates and infants

BACKGROUND Early complete repair of truncus arteriosus with homograft reconstruction of the right ventricular outflow tract was performed and long-term results were evaluated. METHODS Review of 46 consecutive patients with truncus arteriosus who underwent primary correction between June 1987 and May 1997 was performed. Ages ranged from 21 days to 7.2 years (median, 62 days) and weights from 1.8 to 21.5 kg (median, 3.4 kg). Ten patients were operated on before 1 month of age, 20 between 1 and 3 months, 13 between 3 months and 1 year, and 3 at an older age. Associated cardiac conditions were encountered frequently, the most common being coronary artery anomalies (n = 16), truncal valve incompetence (n = 12), and interrupted aortic arch (n = 5). The right ventricular outflow tract was reconstructed with an aortic (n = 28) or a pulmonary homograft (n = 18). RESULTS There were two hospital deaths (4.3%). Both patients had severe truncal valve regurgitation and interrupted aortic arch together with other unfavorable conditions. Survival in uncomplicated truncus arteriosus was 100%. Follow-up was from 3 months to 10 years (mean, 36 months). There was one late death 4 months after the initial repair, presumably because of cardiac reason. Actuarial survival was 93% at 4 months and beyond. Actuarial freedom of reoperation in the 27 hospital survivors with aortic homografts was 43% at 75 months; it was 73% at 62 months in the 17 patients surviving with pulmonary homografts. CONCLUSIONS Neonatal or early infancy complete repair is the treatment with the best potential for survival. The homograft remains our conduit of choice to establish continuity between the right ventricle and the pulmonary artery. Management of severe truncal valve incompetence remains a surgical challenge.

Results of Arterial Switch Operation for Primary Total Correction of the Taussig-Bing Anomaly

BACKGROUND This study evaluates the results of the arterial switch operation for early total repair of double-outlet right ventricle with subpulmonary ventricular septal defect (the Taussig-Bing heart). METHODS From 1986 through April 2003, 27 patients with Taussig-Bing anomaly underwent arterial switch operation. Twenty patients were neonates (n = 11) or infants younger than 3 months (n = 9). Obstruction of aortic arch (n = 19) or subaortic right ventricular outflow tract obstruction (n = 20) and unusual coronary artery patterns (n = 19) were common. Total correction as a single procedure was performed in 21 patients. Events are depicted by Kaplan-Meier curves. RESULTS There was 1 patient hospital death at 2 months after repair. One patient died late that was not cardiac related. Survival was 92% +/- 6% at 8 months and remained constant thereafter. Four patients underwent reoperation (1 for residual aortic arch obstruction and 3 for subvalvular and valvular pulmonary stenosis). Freedom from reoperation decreased to stabilize at 83% +/- 8% after 2 years. The risk to have right ventricular outflow tract obstruction develop was 33% +/- 10% at 1 year, increasing slowly and leveling out at 57% +/- 12% at year 5 and thereafter. Statistical analysis revealed no significant risk factor for death or need for reoperation. CONCLUSIONS The Taussig-Bing anomaly should be corrected in the neonatal period or in early infancy by arterial switch operation, closure of the ventricular septal defect, and simultaneous correction of associated cardiovascular anomalies as a one-stage procedure. Right ventricular outflow tract obstruction often complicates the postoperative course and is the main cause for reintervention.

Congenital pulmonary sling, aorto–pulmonary window and pulmonary vein obstruction as a diagnostic and therapeutic challenge in an infant with VACTERL association

SummaryIn a newborn with anal atresia and urethral valves an incomplete VACTERL association, was diagnosed and a colon anus praeter was placed. Sweating and heart murmur led to cardiac diagnostics. By 2D colour Doppler echocardiography a huge atrial septal defect and pulmonary venous stenoses were diagnosed. Additionally, a pulmonary sling combined with an aortopulmonary window (AoPAw) was suspected and later confirmed by angiography. Heart failure developed and closure of the AoPAw, transplantation of the left pulmonary artery and closure of the atrial septal defect was performed at the age of 4 weeks. But the patient did not improve. Pulmonary hypertension with suprasystemic pressure in the right ventricle originating from a stenosis of the new orifice of the left pulmonary artery and obstruction of the right pulmonary veins was diagnosed by echocardiography and confirmed by angiography.At the age of 8 months the orifice of the left pulmonary artery was enlarged by a patch, the obstruction of left sided pulmonary veins was opened, and the rightsided pulmonary veins were newly implanted into the left atrium. Finally the inter–atrial communication was closed by a patch plastic. Again, postoperatively the patient improved only slightly. At the age of 10 months trans–septal catheterisation was performed. Angiography revealed a successful balloon dilatation of the long obstruction of the right pulmonary veins. Later on, an absorbable magnesium stent was implanted into the right upper pulmonary vein and medication with an endothelin antagonist was started. Temporarily the patient improved significantly. Within 6 weeks, right ventricular systolic pressure was again suprasystemic due to extreme inflow obstruction of the right pulmonary vein diagnosed by echocardiography. Severe heart failure developed and at the age of 1 year the patient died.

Emergency Starnes Operation: A Surgical Success Beyond Any Better Expectation

The wide anatomic spectrum of Ebsteins anomaly is reflected by its extremely variable clinical presentation. We report a case of severe Ebsteins anomaly with natural history of a boy who underwent a successful emergency modified Starnes operation in a Third World sub-Saharan African country during a charitable international surgical mission. The particularities of this case are represented by the modality and setting of the intervention and by a surprising and fast clinical recovery. The age of the patient at the intervention and the choice to not fenestrate the patch also represent the noteworthiness of this life-saving procedure.

Systolisch-diastolisches Herzgeräusch und linksventrikuläre Hypertrophiezeichen

Im Rahmen der Verdachtsdiagnose „muskulärer Ventrikelseptumdefekt“ (VSD) waren bei einem knapp 9-jährigen Patienten jahrelang Kontrolluntersuchungen durchgeführt worden. Zu keiner Zeit bestand eine klinisch relevante Herzinsuffizienz. Das Kind war stets gut belastbar. Auffällig war lediglich eine verstärkte Neigung zum Schwitzen. Echokardiographisch zeigten sich zudem eine Erweiterung des linken Ventrikels sowie eine durch den VSD nicht erklärbare linksventrikuläre Myokardhypertrophie. Hinsichtlich angeborener Herzfehler bestand eine unauffällige Familienanamnese.