Surgical repair of coronary artery ostial stenosis in patients with Williams and elastin arteriopathy syndromes.
The Journal of thoracic and cardiovascular surgery
Surgical repair of peripheral pulmonary artery stenosis in patients without williams or alagille syndromes.
Seminars in thoracic and cardiovascular surgery
OBJECTIVE: Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest. The purpose of this study was to review our experience with the surgical repair of CAOS in patients with Williams and elastin arteriopathy syndromes.METHODS: This was a retrospective review of 16 patients with Williams (n=11) or elastin arteriopathy (n=5) who underwent surgical repair of CAOS as a concomitant procedure with SVAS and/or PPAS repair. Eleven patients had moderate or severe SVAS, and 10 had PPAS. The median age at surgery was 9months (range, 3-108months).RESULTS: Seven patients had repair of the left main and right coronary ostia, 6 the left main, and 3 the right coronary ostium. Median duration of aortic crossclamp was 56minutes and cardiopulmonary bypass time was 454minutes. The median SVAS gradient decreased from 70 to 12mm Hg and pulmonary artery pressure decreased from 120 to 30mm Hg. There was 1 operative mortality (6%). The remaining 15 patients were discharged from the hospital and are alive at a median of 17months' follow-up.CONCLUSIONS: The data demonstrate that patients with Williams and elastin arteriopathy syndromes presenting with SVAS and/or PPAS plus CAOS can undergo successful repair of all hemodynamic issues simultaneously.
View details for DOI 10.1016/j.jtcvs.2020.08.070
View details for PubMedID 32977964
Peripheral pulmonary artery stenosis (PPAS) is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included: supravalvar aortic stenosis (n=8), tetralogy of Fallot (n=4), d-transposition of the great arteries (n=2), truncus arteriosus (n=2), hypoplastic left heart syndrome (n=2), ventricular septal defect (n=1), and patent ductus arteriosus (n=1). Additional medical diagnoses in 15 patients (50%) included: elastin arteriopathy (n=9), pulmonary artery calcinosis (n=1), arterial tortuosity syndrome (n=1), DiGeorge syndrome (n=1) and Noonan syndrome (n=1). Median age at surgery was 3.6 years (IQR 1.6-7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95±0.2 to 0.28±0.08 (p<0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios.
View details for DOI 10.1053/j.semtcvs.2020.01.003
View details for PubMedID 31958550