Síndrome de Hedinger / Hedinger syndrome

Resumen La afectación cardíaca por tumor carcinoide (síndrome de Hedinger) sigue siendo una causa importante de morbilidad y mortalidad entre los pacientes con síndrome carcinoide y tumores neuroendocrinos metastásicos. El estudio de imagen inicial ante la sospecha clínica es el ecocardiograma transtorácico, el cual es importante para la detección temprana de la afectación cardíaca. La cirugía cardíaca, en casos apropiados, es la única terapia definitiva para el tumor carcinoide con afectación cardíaca y mejora los síntomas del paciente y la supervivencia. El tratamiento es complejo y multidisciplinario; el estado cardíaco, el síndrome hormonal y la carga tumoral son fundamentales para guiar el momento óptimo de la cirugía. Se presenta el caso de una paciente joven, con síndrome de Hedinger con afectación valvular tricúspide y pulmonar, quien recibió tratamiento quirúrgico y tuvo evolución exitosa.

Abstract Cardiac carcinoid tumor involvement (Hedinger's syndrome) remains a major cause of morbidity and mortality among patients with carcinoid syndrome and metastatic neuroendocrine tumors. The initial imaging study in case of clinical suspicion is the transthoracic echocardiogram, which is important for the early detection of cardiac involvement. Cardiac surgery, in appropriate cases, is the only definitive therapy for carcinoid tumor with cardiac involvement and improves patient symptoms and survival. Treatment is complex and multidisciplinary; cardiac status, hormonal syndrome, and tumor burden are essential to guide the optimal timing of surgery. The case of a young female patient with Hedinger's syndrome with tricuspid and pulmonary valve involvement, who received surgical treatment and had a successful evolution.

Mid-term outcomes in patients undergoing tricuspid valve repair with rheumatic tricuspid valve disease / 中华胸心血管外科杂志

Objective The aim of this study was to summarize the characteristics of rheumatic tricuspid valve disease ( RTVD) and to evaluate the mid-term outcomes in patients undergoing tricuspid valve repair with RTVD. Methods Between January 2009 and June 2016, 251 consecutive patients with rheumatic heart disease( RHD) underwent left-sided valvular re-placement by a single surgeon. We analyzed 39 patients with RTVD which was diagnosed during the operation. Among them, 32 patients, with moderate or higher tricuspid regurgitation( TR) , were compared with other 59 patients of functional tricuspid regurgitation( FTR) for a better understanding of the features of the RTVD. A total of 39 patients were categorized into 2 groups:Ring annuloplasty group(n=33) and non-Ring annuloplasty group(n=6) which consisted of modified De Vega annu-loplasty for 4 patients and edge-to-edge repair for 2. Meanwhile, 13 of them underwent concomitant tricuspid commissurotomy and 1 patient had a tricuspid leaflet augmentation procedure. We analyzed the mid-term outcomes of 22 patients( follow-up du-ration>1 year)with a mean follow-up duration of(45.5 ±25.1) months. Results Compared with FTR, patients with RTVD had higher preoperative TR grade(3.1 ±0.8 vs. 2.6 ±0.7, P=0.004) but with lower preoperative PASP[(53.8 ±19.4) mmHgvs.(63.6±21.5)mmHg,P=0.037)](1mmHg=0.133kPa) andtricuspidannulusdiameter(TAD) thatobserved bothinpreoperativeechocardiogramtests[(37.0±5.7)mmvs.(41.9±6.7)mm,P=0.018)]andintraoperativedetection [(35.6±4.1)mmvs.(39.9±6.5)mm,P=0.000)] . TherewasnoearlymortalityandresidualmoderateorhigherTR grades in either group. Compared with patients in non-ring annuloplasty group, patients in ring annuloplasty group showed low-er postoperative TR grade(0. 2 ± 0. 4 vs. 0. 7 ± 0. 5, P=0. 039) and acceptable TR grade(0. 8 ± 0. 5 vs. 1. 3 ± 1. 9, P>0. 050) during the mid-term follow-up. PASP, the peak diastolic velocity and pressure gradient across tricuspid valve were not different between groups in preoperative, postoperative and follow-up. Conclusion Compared with FTR, Patients with RTVD had lower preoperative PASP and TAD, but with a higher preoperative TR grade. In our study, ring annuloplasty showed simi-lar mid-term outcomes compared with other procedures.

Aseptic Endocarditis in Behcet's Disease Presenting as Tricuspid Valve Stenosis

Aseptic endocarditis is an uncommon complication of Behcet's disease (BD). We describe a rare case of a 39-year-old female who had BD with aseptic endocarditis of the tricuspid valve (TV) presenting as tricuspid stenosis. She was diagnosed with BD four years ago. The mucocutaneous lesions were well-controlled with colchicine and short courses of corticosteroids. She remained free of signs and symptoms of BD for one year without any medication. Three months before admission, she gradually developed dyspnea on exertion and peripheral edema. Echocardiography revealed dilated right atrium and markedly thickened TV with severe stenosis. TV replacement was performed. Pathologic examination of the valve showed fibrinoid necrotic material and inflammatory cell infiltration. Blood cultures and cultures of the excised valve were negative for microorganisms.

Severe tricuspid stenosis related to endocardial pacemaker lead / Estenose tricúspide severa induzida por eletrocatéter de marcapasso

Tricuspid stenosis (TS) is an uncommon complication of transvenous ventricular pacing. We present a case of TS developed 14 years after pacemacker implantation. The etiology of stenosis appeared to be inflammation and fibrosis of tricuspid valve (TV) secondary to leaflet perforation by the pacemacker lead and it was managed successfully with surgical valvuloplasty. Iatrogenic TS may occur more frequently than is clinically suspected and it should be considered in any patient with endocardial leads presenting with right heart failure.