Aortic dissection in a patient with a dilated aortic root following tetralogy of Fallot repair

Autor: Jasmine Grewal, Rekha Raju, Vishva A. Wijesekera, Rudy Chow, Marla Kiess, Amanda J. Barlow, Jonathon Leipsic
Rok vydání: 2014
Předmět:
Zdroj: International Journal of Cardiology. 174:833-834
ISSN: 0167-5273
Popis: With improved longevity, aortic root dilation has been increasingly recognized among patients with tetralogy of Fallot (TOF). Aortic dissection is thought to be rare with only three previous cases [1,3,2] reported in the literature. We describe the case of a patient who presented following aortic root dissection late after TOF repair. This 60 year old male had TOF repair at age 14, and was known to have a dilated aortic root. Infrequent (five yearly) transthoracic echocardiograms from 1999 to 2011 showed no progressive aortic root dilation although the ascending aorta was not always well visualized. There was no significant residual valvular disease, outflow tract obstruction or ventricular septal defect. Computed tomography (CT) of the aorta in January 2012 measured a transsinus diameter of 53 mm (24 mm/m; normal b20 mm/m) and proximal ascending aorta diameter of 49 mm which were similar to measurements obtained on an ascending aortogram a few months later. Apart from being obese (height 173 cm, weight 100 kg), he had no comorbidities and was on no regular medications. He presented in April 2013 with a two week history of new onset class III-IV dyspnea that was preceded by an episode of acute chest pain lasting three hours. Clinically he had left ventricular failure with a loud aortic regurgitant murmur, bilateral pleural effusions and a serum BNP 1400 ng/L (normal b40 ng/L). A CT of the aorta (Fig. 1) showed a trans-sinus diameter of 55 mm but there was now a limited dissection involving the right coronary cusp causing severe aortic regurgitation confirmed by echocardiography. He underwent a Bentall operation. Various hypotheses have been proposed for the mechanism of aortic root dilatation in this group of patients including: increased flow through the aorta due to right to left shunting before repair [4]; embryological defects of spiral septum growth leading to a larger aortic diameter at the expense of the pulmonary artery [5]; and an intrinsic aortopathy related to cystic medial degeneration [5]. The prevalence of aortic root dilation in TOF patients has been reported in a few studies and ranges between 15 and 87% depending on the definitions used [6], and when defined as an observed-to-expected ratio indexed to body surface area and age, was only present in 6.6% of patients [6]. In this case, the indexed observed-to-expected aortic dimension was increased at 1.58. In the previous three reports of aortic dissection in repaired TOF, the aortic dimensions were 71 mm [1], 93 mm [3] and 70 mm [2]; significantly larger than in this case. There is no consensus as to how TOF patients with aortic root dilation should be managed. Intervention when the ascending aorta reaches 55 mm [4] has been suggested as aortic dilatation is associated with increased stiffness and hence increased risk of aortic dissection [7]. The low number of reported cases may suggest
Databáze: OpenAIRE
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