Postpneumonectomy Compression of the Mitral Annulus: Rare Vascular Complication in Sportive Patient.

Numerous postpneumonectomy complications exist. We present a rare clinical case of postpneumonectomy exertional dyspnea revealing compression of the mitral annulus by the descending aorta. The patient was 42-year-old former smoker with pulmonary emphysema. He has been operated on, in 2012 (i.e., right pneumonectomy). Before the surgery, the patient was a recreational runner. However, after some months, it was difficult for the patient to resume running. Cardiopulmonary exercise testing indicated moderate exercise intolerance with important oxygen desaturation. More interestingly, a decrease of low oxygen pulse was noticed from the first ventilatory threshold with no electrical modification on the electrocardiogram. This decrease was indicative of a decline in stroke volume. The thoracic scan revealed a right pneumonectomy pocket with a liquid abnormal content. Moreover, the mediastinum had shifted toward the pneumonectomy space and the left lung was distended and emphysematous. Echocardiography revealed a major change in the mediastinal anatomy. The mitral annulus was observed to be compressed by the rear wall of the descending aorta. The diagnosis of postpneumonectomy syndrome or platypnea-orthodeoxia syndrome was ruled out in this patient. Mitral annular compression by the descending aorta is rare complication, which must be researched in patients with postpneumonectomy exertional dyspnea.

Patients with severe aortic stenosis and reduced ejection fraction: earlier recovery of left ventricular systolic function after transcatheter aortic valve implantation compared with surgical valve replacement.

BACKGROUND: Only 50% of patients with aortic stenosis (AS) and low ejection fraction (EF) improve their contractility after surgical aortic valve replacement (AVR). Long-term prognosis of these patients is strongly correlated to EF recovery after the surgery. The aim of this study was to compare the postoperative left ventricular function recovery in patients with severe AS and reduced EF after AVR and transcatheter aortic valve implantation (TAVI). METHODS AND RESULTS: Twenty-one patients undergoing AVR for severe AS (<1 cm²) and reduced EF (<45%) were echocardiographically compared with 31 patients treated with TAVI by conventional and two-dimensional (2D) strain method. TAVI patients were older (83 ± 6 vs. 74 ± 13 years, P < 0.001), had more comorbidities (Euroscore 26 ± 15% vs. 16 ± 12%, P < 0.001) and no differences in ischemic heart disease (16.1% vs. 9.5%, P = 0.39). EF was similar in both groups at baseline. Aortic valve area significantly increased (P = 0.01) while pressure gradient decreased (P = 0.009) after both procedures but TAVI was associated with a better EF recovery compared with AVR at day 7. Regional function assessed by radial 2D strain increased in TAVI group but was transiently deteriorated in AVR patients (P < 0.05). Longitudinal as well as circumferential 2D strain progressively increased at follow-up in both groups (P < 0.05). EF was similar in both groups at 3-month follow-up. CONCLUSION: In patients with severe AS and reduced EF, TAVI is associated with faster and better recovery of LV function compared with AVR.

De novo heterozygous desmoplakin mutations leading to Naxos-Carvajal disease.

STUDY/PRINCIPLES: Arrythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal-dominantly inherited disease caused by mutations in genes encoding desmosomal proteins and is characterised by fibrofatty replacement occurring predominantly in the right ventricle and can result in sudden cardiac death. Naxos and Carvajal syndrome, autosomal recessive forms of ARVC/D, are characterised by involvement of the right and/or left ventricle in association with palmoplantar keratoderma and woolly hair. The aim of the present study has been to screen for mutations in the desmosomal protein genes of two unrelated patients with Naxos-Carvajal syndrome. METHODS AND RESULTS: Desmosomal protein genes were screened for mutations by polymerase chain reaction as well as direct sequencing approach. In each patient we identified a single heterozygous de novo mutation in the desmoplakin gene DSP, p.Leu583Pro and p.Thr564Ile, leading to severe combined cardiac/dermatological and cardiac/dermatological/dental phenotypes. The DSP missense mutations are localised in the N terminal domain of desmoplakin. CONCLUSION: The identified variations in DSP involve highly conserved residues. Moreover, the variations are de novo mutations and they are localised in critical protein domains that appear to be mutation hot spots. We assume that these heterozygous variations are causal for the mixed Naxos-Carvajal syndrome phenotype in the screened patients.

Significant mitral regurgitation regression by slight modification of the right ventricular pacing site.

A dual-chamber pacemaker was implanted because of symptomatic bradycardia in a 60-year-old patient free from other heart disease. Then, the patient developed acute congestive heart failure due to pacing-induced mitral regurgitation (MR). Under echocardiographic guidance, modification of location of the right ventricular pacing lead led to a significant decrease in MR severity resulting in immediate clinical improvement.