During a 20-year period, between June 1987 and May 2007, 43 consecutive patients presented to Children’s Hospital, Denver, with mitral stenosis and multiple levels of left heart obstruction. Patient records, including operative reports, diagnostic reports, inpatient records, and outpatient clinic visit notes were reviewed in accordance with an approved protocol from the Colorado Multiple Institutional Review Board. Individual patient or parental consent was waived because of the retrospective nature of the study. Patients requiring a single-ventricle management pathway were excluded. Echocardiography was used to characterize the cardiovascular anatomy in all patients. In patients who underwent transplantation, additional anatomic data were obtained from pathologic examination of the explanted heart.
The SR group comprised 30 patients (17 boys, 13 girls) with mitral stenosis and multiple left-sided obstructive lesions who underwent a staged reparative approach. In 27 patients (90%), the initial operation was performed when they were aged younger than 1 year.
The TX group comprised 13 patients (7 boys, 6 girls) with Shone’s complex who were listed for transplantation. The median age at first operation was 22 days (range, 2 to 76 days). Of these, 11 (85%) survived to transplantation. Median age at transplantation was 5 months (range, 1 to 20 months).
Mitral Valve Morphology
The specific mitral lesions are summarized in . All patients had structural abnormalities of the mitral valve. A mean gradient across the left ventricular inflow greater than 6 mm Hg was present in 37 children (86%). Parachute deformity of the mitral subvalvar apparatus was present in 15 (35%), and a supravalvar mitral ring was present in 13 (30%). Severe deformities of the subvalvar apparatus such as the hammock or arcade malformations, as described by Uva and colleagues [3
] with a combination of papillary muscle hypertrophy and foreshortened chordae, were found in 14 patients (33%).
Spectrum of Left-Sided Heart Inflow Lesions
Left-Sided Outflow Obstructions
Left-sided heart obstructive lesions are reported in . Coarctation of the aorta was the most prevalent left-sided obstructive lesion and was present in 38 of 43 patients (88%). Hypoplasia of the aortic arch was present in 20 (47%) with coarctation. Significant sub-aortic stenosis affected 25 patients (58%). Important valvar aortic stenosis developed in 24 patients (56%), supravalvar stenosis occurred in 3 (7%), and an associated ventricular septal defect was present in 20 (47%).
Distribution of Left-Sided Outflow Obstructive Lesions
In the SR group, 30 patients underwent a total of 72 operations. There were 31 left ventricular inflow procedures in 22 patients. Twenty-seven patients underwent 56 procedures to address outflow obstructive lesions.
The median age at first operation was 6.5 days (range, 1 day to 1.6 years). Coarctation repair was the initial operative procedure in 24 of 30 patients (80%). Aortic arch hypoplasia was addressed in 5 patients undergoing coarctation repair. Other procedures performed at time of coarctation repair included pulmonary artery banding (PAB) in 4 and aortic valvotomy in 2. Other procedures performed at the initial presentation include subaortic resection in 2, Ross-Konno and mitral valve repair (MVP) in 1, Ross-Konno and mitral valve replacement (MVR) in 1, supraannular mitral ring resection in 1, and aortic valvotomy in 1.
A second surgical procedure was performed in 25 of 30 patients (83%). The median age at the second operation was 11 months (range, 1 month to 10.8 years). The median interval between the first and second operation was 10.4 months (range, 24 days to 10.6 years). Procedures performed at the second operation included MVP in 10, ventricular septal defect closure in 8, subaortic membrane resection in 7, aortic valvotomy in 4, supraannular mitral ring resection in 7, Ross-Konno in 2, and 1 each with extended homograft aortic root replacement, PAB, and heart transplantation.
A third operation was required in 11 patients (37%) at a median age of 1.9 years (range, 1.0 to 9.5 years). The median interval between the second and third operations was 12.7 months (range, 4.2 months to 5.7 years). Procedures performed at the third operation include subaortic membrane resection in 4, Konno-aortic valve replacement (AVR) in 3, MVP in 2, MVR in 2, and in 1 patient each, Ross-Konno procedure, Ross II procedure, ventricular septal defect closure, and extended homograft aortic root replacement.
A fourth operation was required in 4 patients (13%). The median age at the fourth procedure was 6.3 years (range, 2.9 to 13.3 years), during which four MVRs and one Konno-AVR were performed. A fifth operation was performed in 1 patient (3%), who received a heart transplant at 3.7 years.
In the TX group, 13 patients underwent 26 procedures. Coarctation repair was performed in 10 patients as the initial operation at a median age of 15 days (range, 2 to 74 days). Primary coarctation balloon angioplasty was performed in 2 additional patients. Concomitant procedures included PAB in 4 and aortic arch augmentation in 1. Another patient underwent a PAB as the sole initial procedure. One patient underwent MVR at 6 months while on the waiting list. Heart transplantation was successfully performed in 11 patients (85%) at a median age of 5 months (range, 1 to 23 months). The median time on the waiting list was 73 days (range, 5 to 297 days).
Statistical analysis was performed with Prism 5.0 software (GraphPad Inc, San Diego, CA). Data are described as median with ranges, or mean with standard deviation. Serial data were compared between groups using the Student t test and Fisher exact test. Risk factors were assessed using log-rank analysis. Survival and freedom from reoperation were determined by actuarial Kaplan-Meier analysis. Values of p < 0.05 were considered statistically significant.