We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.
Objective(s) To determine the influence of surgical techniques adopted to avoid suture line disruption, periprosthetic leakage, patch dislodgement, pericardial patch aneurysm formation, and the long-term stability of aortic root enlargement (ARE) during aortic valve replacement (AVR). Methods One hundred fifteen patients undergoing AVR or combined aortic and mitral valve replacements with Nicks' posterior ARE between 1997 and 2019 underwent long-term echocardiographic and angio-computed tomographic evaluation. Age was 11-72 years (AVR: median, 30; interquartile range, 21-47 years; AVR and mitral valve replacement: median, 27.5; interquartile range, 20-37.5 years). The aortotomy was closed using autologous pericardial patch and Teflon-buttressed sutures. Results Hospital mortality was 1.7% (n = 2), with 4 (3.5%) late deaths. At a mean follow-up of 123.11 ± 77.67 months, the survival probability from Kaplan–Meier was 93.25 ± 0.03%. No cases of severe prosthesis–patient mismatch (PPM) were observed, and only 2 patients had moderate PPM. Median aortic root diameters at the level of sinus of Valsalva and sinotubular junction were 32 (29-35) mm and 33 (30-36) mm, respectively, at discharge, and were 33 (30-36) mm, and 33 (31-37) mm, respectively, at latest follow-up, with no cases of late pericardial patch aneurysm. Conclusions ARE is a safe adjunct to AVR in patients with a small aortic annulus to prevent PPM. Retention of a pericardial collar and Teflon-buttressed sutures is an expedient, safe, and effective technique in reducing bleeding at the enlarged ventriculo-aortic junction. Autologous pericardial patch aortoplasty is not associated with late aneurysm/pseudoaneurysm formation.
The present perspective is a synthesis of published investigations in the setting of subvalvular aortic aneurysms. We identified 75 investigations and reviewed the clinical presentation, diagnostic modalities used, surgical techniques employed, and their outcomes. Clinical presentation, radiographic findings, transthoracic and transesophageal echocardiography, electrocardiogram-gated computerized tomography, and magnetic resonance imaging provided the diagnostic information and were used to define the disease entity before surgery. In this article, we have attempted to address several issues concerning establishment of diagnosis, varied clinical presentation, and their management. We submit that an increased appreciation of this disease entity will contribute to improved surgical management.
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