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Aortic root surgery in Marfan syndrome.

ABSTRACT: Marfan syndrome (MFS) is an autosomal dominant connective disease etiologically related with FBN-1 gene mutation. The altered microfibril protein structure result in characteristic cardiovascular abnormalities including aortic root dilatation, aortic root aneurysms, and aortic dissections. Aortic root aneurysms and subsequent dissection are the major causes of reduced life expectancy in MFS patients. Prophylactic aortic root replacement has improved the survival of patients with MFS. Elective root replacement carries very low mortality and morbidity. Emergency root replacement for dissection is accompanied with higher early mortality and late deaths/interventions. Both the techniques of aortic root replacement, total root replacement (TRR) with a prosthetic valved conduit, and valve-sparing root replacement (VSRR) yield excellent early and late results. Considering the low risk of prosthetic valve-related events, the improved long-term survival, and event-free survival, TRR continues to be a very effective surgical option. VSSR also seems to be a good option for the first 10 to 15 years following surgery in MFS patients. The choice of procedure depends upon available expertise, patients' choice, feasibility of anticoagulation, possibility of pregnancy, and lifestyle of the patient. Aortic root morphology and state of valve cusps also affect the decision making. In recent times, personalized external aortic root support (PEARS) with a macroporous mesh sleeve has also emerged as a promising alternative to aortic root replacement. All these patients need close monitoring for whole life after surgical intervention.

SUBMITTER: Choudhary SK

PROVIDER: S-EPMC7525511 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Aortic root surgery in Marfan syndrome.

Choudhary Shiv Kumar SK Goyal Aayush A

Indian journal of thoracic and cardiovascular surgery 20181203 Suppl 2

Marfan syndrome (MFS) is an autosomal dominant connective disease etiologically related with <i>FBN</i>-<i>1</i> gene mutation. The altered microfibril protein structure result in characteristic cardiovascular abnormalities including aortic root dilatation, aortic root aneurysms, and aortic dissections. Aortic root aneurysms and subsequent dissection are the major causes of reduced life expectancy in MFS patients. Prophylactic aortic root replacement has improved the survival of patients with MF ...[more]

PMID: 33061070

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Project description:Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3. Repeated measures linear and logistic regressions were used to determine multivariable predictors of AoR dilation. Receiver operator characteristic curves were used to determine cut-points in AoR dilation predicting referral for aortic surgery. Multivariable analysis showed rapid AoR dilation as defined by change in AoRz/year > 90th percentile was associated with older age, higher sinotubular junction z-score, and atenolol use (R2 = 0.01) or by change in AoR diameter (AoRd)/year > 90th percentile with higher sinotubular junction z-score and non-white race (R2 = 0.02). Referral for aortic root surgery was associated with higher AoRd, higher ascending aorta z-score, and higher sinotubular junction diameter:ascending aorta diameter ratio (R2 = 0.17). Change in AoRz of 0.72 SD units/year had 42% sensitivity and 92% specificity and change in AoRd of 0.34 cm/year had 38% sensitivity and 95% specificity for predicting referral for aortic surgery. In this cohort of young patients with MFS, no new robust predictors of rapid AoR dilation or referral for aortic root surgery were identified. Further investigation may determine whether generalized proximal aortic dilation and effacement of the sinotubular junction will allow for better risk stratification. Rate of AoR dilation cut-points had high specificity, but low sensitivity for predicting referral for aortic surgery, limiting their clinical use. Clinical Trial Number ClinicalTrials.gov number, NCT00429364.

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Aortic root surgery in Marfan syndrome.

Publications

Aortic root surgery in Marfan syndrome.

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