Multicentre experience with valve-sparing aortic root replacement by means of combined remodelling and external aortic ring annuloplasty in patients with Marfan syndrome.

OBJECTIVES: The most recent valve-sparing root replacement technique combines the advantages of the reimplantation (David) and remodelling (Yacoub) techniques. The aortic root is reconstructed according to the remodelling technique, the aortic valve is repaired according to the principle of effective height, and an external ring provides annular support. The purpose of this study was to evaluate operative and mid-term outcomes using this technique in patients with Marfan syndrome. METHODS: Adult patients with Marfan syndrome who had an indication for aortic root surgery according to European Society of Cardiology guidelines and were operated on using this new root replacement technique were retrospectively evaluated. Follow-up was obtained from standard outpatient visits and included echocardiography. RESULTS: The study group comprised 22 patients (mean age 36 years, 68% males). Mean follow-up was 7.5 years. There were no mortalities. Two patients required aortic valve replacement because of aortic regurgitation. In both patients, the aortic root was severely dilated (≥65 mm) preoperatively, with grade III aortic valve regurgitation and aortic valve cusps that were very fragile. Aortic regurgitation was grade ≤I on follow-up in 18 of the remaining 20 patients. CONCLUSIONS: Valve-sparing root replacement using remodelling combined with aortic-ring annuloplasty is safe in patients with Marfan syndrome. The mid-term outcome is promising in patients undergoing elective valve-sparing root replacement at recommended root diameters. However, in patients with extremely dilated aortic roots and already severe aortic regurgitation, the technique should be used cautiously as aortic cusps are fragile and might not be suitable for durable repair. CLINICAL REGISTRATION NUMBER: UMCG Research registry #11208.

Surgical Repair of a Sinus of Valsalva Aneurysm.

A surgically challenging case of an unruptured Sinus of Valsalva aneurysm (SoVA) with severe aortic regurgitation (AR) due to cusp prolapse is presented. Sinus reconstruction with a patch cut out from the sinus portion of a Gelweave Valsalva graft (Terumo Vascutek) was performed. Intraoperative measurements showed insufficient effective height of the right coronary cusp; therefore, cusp plication and pericardial patch augmentation of the right coronary cusp were performed with satisfying result.

Reference Values for Physical Stress Echocardiography in Asymptomatic Patients after Mitral Valve Repair.

BACKGROUND: Clinical decision-making in symptomatic patients after mitral valve (MV) repair remains challenging as echocardiographic reference values are lacking. In native MV disease intervention is recommended for mean transmitral pressure gradient (TPG) >15 mmHg or systolic pulmonary artery pressure (SPAP) >60 mmHg at peak exercise. Insight into standard stress echo parameters after MV repair may therefore aid to clinical decision-making during follow-up. HYPOTHESIS: Stress echocardiography derived parameters in asymptomatic patients after successful MV repair differ from current guidelines for native valves. MATERIAL AND METHODS: In 25 patients (NYHA I) after MV repair stress echocardiography was performed on a semi-supine bicycle. Doppler flow records and MV related hemodynamics at rest and peak were obtained. Linear regression analysis was performed for mean TPG and SPAP at peak, using predetermined variables and confounders. RESULTS: Mean TPG at rest (3.2 ± 1.4 mmHg) significantly increased at peak (15.0 ± 3.4 mmHg) but was always <25 mmHg. Mean SPAP at rest (21.4 ± 3.8 mmHg) significantly increased at peak (41.8 ± 8.9 mmHg) but was never >57 mmHg. Only the indexed MV ring diameter was inversely correlated to mean TPG at peak in a multivariable model. CONCLUSION: In contrast to current recommendations in native MV disease, our data indicate that the standard value for mean TPG during stress echocardiography in asymptomatic patients after successful MV repair was above the guideline threshold of 15 mmHg in >50%, but always <25 mmHg. For SPAP, patients never reached the guideline cutoff (60 mmHg). Long-term follow-up data are needed to provide insight in clinical consequences. Baseline stress echocardiography may indicate individual reference values to compare with during follow-up. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT02371863?term=chamuleau+AND+Mitral&rank=1.

Giant left atrial appendage: a rare anomaly.

A giant left atrial appendage is a rare congenital anomaly that has been reported on only a few occasions. We report two symptomatic patients with atrial fibrillation combined with a cerebellar infarct in one and dyspnea in the other. Both patients were treated surgically with resection of the giant left atrial appendage and radiofrequency pulmonary vein isolation. Recognition of this uncommon pathology can lead to timely surgical intervention.

Reduced connexin40 protein expression in the right atrial appendage of patients bearing the minor connexin40 allele (-44 G --> A).

AIMS: The occurrence of connexin40 (Cx40) minor polymorphism (-44 G → A) was increased in patients with idiopathic atrial fibrillation (AF), although its effect on atrial Cx40 protein expression is unknown. We aimed to evaluate whether alterations in Cx40 are directly linked to the development of AF, we studied the effect of this polymorphism on Cx40 expression and distribution in patients without any history of AF and in patients who developed post-operative AF. METHODS AND RESULTS: Hundred and eight patients (mean age 67 ± 9 years), without a history of AF or conditions that predispose to AF, were included. During heart surgery, 10 cc blood was collected for DNA genotyping and the right atrial appendage was partly excised. Ten patients (9%) were homozygous for the minor allele (AA, Group 1), 30 (28%) were heterozygous (AG, Group 2), and 68 (63%) were non-carriers (GG, Group 3). Ten age- and sex-matched tissue samples per group were analysed for Cx40 expression by: (i) real-time quantitative polymerase chain reaction (Q-PCR), (ii) western blotting, and (iii) immunohistochemistry on cryosections. Real-time quantitative polymerase chain reaction showed no significant differences of Cx40 mRNA among the groups. Western blot analysis, however, revealed a reduction in Cx40 protein in Groups 1 (-36.4%) and 2 (-39.5%) as compared with Group 3. Immunohistochemistry confirmed this reduction but indicated an unaltered subcellular distribution of the remaining Cx40. Incidence of post-operative AF (28%) was age-dependent but unrelated to the presence of the polymorphism or fibrosis. CONCLUSION: Presence of the Cx40 minor allele (-44 G → A) results in a uniform down-regulation of right atrial appendage Cx40 protein which was not significantly related to development of post-operative AF.