Kent E. Ward

Pulmonary autograft in children: Realized growth potential☆

Pulmonary autograft replacement of the aortic valve has the potential to remain viable and grow in proportion to the somatic growth of the child. Changes in aortic annulus and sinotubular dimensions were compared early and late postoperatively, and related to normal. Eighty-six children, 0.9 to 21 years, were operated on between 1986 and 1993: 42 had a root replacement, 24 an inclusion cylinder, and 20 a scalloped subcoronary implant. Actuarial survival at 7 years was 96.5% +/- 2.0%. Freedom from reoperation for the pulmonary autograft or the homograft reconstruction of the right ventricular outflow tract was 92% +/- 4%. Freedom from reoperation on the autograft in root replacements was 96% +/- 4%, in the inclusion cylinder was 100%, and in the scalloped subcoronary was 90% +/- 7% (not significant). Aortic annulus and sinotubular junction diameters were compared with normal values predicted by body surface area. In 22 intraaortic implants, early and late postoperative annulus diameter mean Z values are in the normal range. In the 23 root replacements, early annulus diameter was within the normal range, but late Z values were larger than normal (p < 0.02). Intraaortic implant annulus diameter increased proportionally to somatic growth, but the sinotubular junction, which was small, remained small but increased toward normal. In the root replacements, the annulus increased in diameter and became dilated. The sinotubular junction, which was small early, increased and was within the normal range late. Lower operative risk and valve durability without failure suggest improved results with inclusion cylinder technique.(ABSTRACT TRUNCATED AT 250 WORDS)

Cytotoxic mAb from rheumatic carditis recognizes heart valves and laminin

Anti-streptococcal antibodies cross-reactive with N-acetyl-betaD-glucosamine (GlcNAc) and myosin are present in the sera of patients with rheumatic fever (RF). However, their role in tissue injury is not clear. In this study, we show that anti-GlcNAc/anti-myosin mAb 3.B6 from a rheumatic carditis patient was cytotoxic for human endothelial cell lines and reacted with human valvular endothelium and underlying basement membrane. Reactivity of mAb 3.B6 with the valve was inhibited by human cardiac myosin > laminin > GlcNAc. The mAb 3.B6 epitopes were localized in fragments of human cardiac myosin, including heavy meromyosin (HMM), the S1 subfragment, and two light meromyosin (LMM) peptides containing amino acid sequences KEALISSLTRGKLTYTQQ (LMM 1) and SERVQLLHSQNTSLINQK (LMM 33). A novel feature of mAb 3.B6 was its reactivity with the extracellular matrix protein laminin, which may explain its reactivity with the valve surface. A laminin A-chain peptide (HTQNT) that includes homology to LMM33 inhibited the reactivity of mAb 3.B6 with human valve. These data support the hypothesis that cross-reactive antibodies in rheumatic carditis cause injury at the endothelium and underlying matrix of the valve.

The Ross Operation in Children: 10-Year Experience

BACKGROUND The Ross operation, first performed in children in 1968, may be the ideal aortic valve replacement. Technical demands of the operation and two valves at risk have delayed acceptance. A review of our experience to assess midterm and long-term results with the Ross operation is presented. METHODS The records of 150 consecutive patients, aged 7 days to 21 years (median age, 12 years, 75% less than 15 years) were reviewed. Follow-up was complete within the last 12 months (median, 2.8 years; range, 1 month to 10 years). Echocardiographic assessment was available on 116 (71%) within 1 year of closure and in 136 (91%) within 2 years. RESULTS Survival was 97.3% at 8 years. Late autograft valve dysfunction required replacement in 2 and reoperation with restitution of autograft function in 6. Freedom from reoperation for autograft dysfunction is 90% +/- 4% at 8 years. Freedom from reoperation for homograft obstruction is 94% +/- 3% at 8 years. Pulmonary homograft dysfunction (gradient > 40 mm Hg) was present in 4 additional patients. Freedom from reoperation on the homograft or a gradient of 40 mm Hg is 89% +/- 4% at 8 years. All patients have a normal, active lifestyle, without anticoagulants for their aortic valve replacement. CONCLUSIONS The Ross operation is the preferred operative replacement in children requiring aortic valve replacement.

A 26-year experience with surgical management of tetralogy of fallot: risk analysis for mortality or late reintervention

BACKGROUND Over the past decade repair of tetralogy of Fallot (TOF) in infancy has gained favor. It is still uncertain what effect early complete repair will have on survival or late reoperation on the right ventricular outflow tract. METHODS To assess these outcomes, we reviewed our experience (1971-1997) with 294 patients undergoing operation at one institution. Median follow-up was 10.6 years (range, 0.1 to 26 years), and was complete for 90.2% patients. RESULTS Primary complete repair was done in 199 patients (68%), and a staged repair in 62 patients (21%). Thirty-three patients had only a palliative procedure. Sixty-eight patients (23.1%) had complex pathologic processes, including pulmonary atresia in 53. Hospital mortality for primary repair was 11.1% (22/199), for staged repair was 17.7% (11/62), and for palliative procedures was 15.5% (16/103 procedures). Since 1990 mortality has been 2.1%, 11.8%, and 0% respectively (p < 0.001), despite younger age at repair (0.6+/-0.1 versus 2.1+/-0.2 years; p < 0.001). Multivariate analysis identified longer period of hypothermic circulatory arrest, pulmonary artery patch angioplasty, earlier year of operation, and closure of the foramen ovale as risk factors for hospital death. For hospital survivors 20-year survival was 98%+/-3% for TOF with pulmonary stenosis and 88%+/-9% for TOF with pulmonary atresia (p=0.09). Reintervention on the right ventricular outflow tract was needed in 14.1% (37/261) patients. Freedom from reintervention on the right ventricular outflow tract at 20 years was 86%+/-4% for TOF with pulmonary stenosis and 43%+/-16% for TOF with pulmonary atresia (p=0.001). For the subgroup TOF with pulmonary stenosis, this was 85%+/-5% after primary repair and 91%+/-8% after staged repair (not significant). At 15-year follow-up, this was 78%+/-10% for patients not older than 1 year at operation compared with 88%+/-4% for older patients (not significant). CONCLUSIONS Early mortality after primary repair of TOF has significantly improved and late survival is excellent. Primary repair in infancy does not increase risk for reintervention on the right ventricular outflow tract.

Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up.

BACKGROUND Ebsteins anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates. We now update our experience with emphasis on the evolution of our surgical technique and the medium-term follow-up of these patients. METHODS Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebsteins anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs. RESULTS One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old. CONCLUSIONS Surgical repair of the severely symptomatic neonate with Ebsteins anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.

Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up.

BACKGROUND Ebsteins anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. METHODS We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebsteins anomaly. CONCLUSIONS Biventricular repair of Ebsteins anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.

Pulmonary autograft replacement in children. The ideal solution

Fifty-one children, aged 1.8 to 21 years (mean, 11.4) with aortic valve replacement using a pulmonary autograft are reviewed. Twenty-nine were intra-aortic implants and 22 were root replacements. There was one operative death, no late deaths, and two have required reoperation. Actuarial freedom from reoperation was 93% +/- 5.5 at 5.6 years. Freedom from progression of aortic insufficiency (AI) was 81% +/- 9 at 5.6 years in the intra-aortic implants and 86% +/- 10 in the root replacement. Enlargement of the pulmonary autograft was seen echocardiographically in both groups. This enlargement was consistent with somatic growth and not associated with progression of AI. Ten of 19 patients with aortic stenosis had an LV mass index suggestive of LV hypertrophy before operation. At 1 year, 18 of 25 had a normal LV mass index. Thirteen of 16 patients with AI had preoperative abnormal LV mass index. All but four returned to normal by 1 year. Low operative risk, excellent function, resolution of abnormal LV hemodynamics, and enlargement consistent with somatic growth suggest that the pulmonary autograft is the ideal replacement for the malfunctioning aortic valve.

Transseptal decompression of the left heart during ECMO for severe myocarditis

A 16-month-old boy suffered a cardiac arrest as a result of acute myocarditis, and venoarterial extracorporeal membrane oxygenation was instituted. Twelve hours later, acute left heart distention developed with cessation of left ventricular ejection. Under transesophageal echocardiographic guidance, a long introducer was placed into the left atrium through a transseptal puncture and connected in-line to the venous circuit. Within hours, left ventricular function improved and ejection returned. Left heart decompression was continued for 5 days, and the patient was weaned from extracorporeal membrane oxygenation after 6 days with normal cardiac and neurologic function.

Medium-term follow-up of pulmonary autograft replacement of aortic valves in children

Pulmonary autograft replacement (PAG) of the aortic valve in children has been shown to be safe and effective with a low incidence of late valve dysfunction. Relief of all types of left ventricular outflow tract obstruction using the pulmonary root has been possible. Concern about the durability of the pulmonary root in the aortic position, and the potential for growth of the pulmonary autograft used either as a root replacement or intraaortic implant, has been questioned. Sixty-five consecutive patients, aged 1.8 to 21 years (mean 12 years) operated on between September 1986 and January 1993, 35 with an intra-aortic implant (IA) and 30 with root replacement (RR), were evaluated by clinical and serial echocardiographic studies (ECHO) up to 6.5 years post-operatively. The hospital mortality rate was 3.0% (70% CL 2.1-5.1%). Two patients required reoperation for PAG insufficiency (AI), one for technical malalignment necessitating replacement at 6 months, and one with progressive leaflet prolapse due to adherence of the valve leaflet to a ventricular septal defect (VSD) patch. Freedom from significant aortic regurgitation at 6-year follow-up was 100% for RR and 91 +/- 6% for IA, and freedom from all valve-related complications including reoperation was 92 +/- 5% at 6 years. Significant enlargement of the aortic annulus which parallels somatic growth has been measured by ECHO in 17 IA implants (P < 0.001) and 17 RR patients (P < 0.01) by 1 year, and in 10 IA (P = 0.007) and 6 RR (P < 0.05) by 2 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Complete repair of Ebstein anomaly in neonates and young infants: A 16-year follow-up

OBJECTIVE The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. METHODS Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. RESULTS Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. CONCLUSIONS Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.