Internacional Journal of Cardiovascular Sciences. 2016;29(2):149-151 149

CASE REPORT

Unusual Form of Tricuspid !

Ana Rita Pereira Alves Ferreira, Sofia Lazaro Mendes, Rui Martins, Silvia Monteiro, Mariano Pego

Centro Hospitalar e Universitário de Coimbra – Hospitais de Universidade de Coimbra – Serviço de Cardiologia A – Coimbra – Portugal

Abstract

Tricuspid stenosis, in rare cases, can be induced by pacemaker electrodes. We report the case of a 72-year-old patient who, in 1982, underwent surgical ablation procedure for Wolf-Parkinson-White syndrome. The procedure was complicated by complete , evolving into permanent pacemaker implantation. In 1992, due to pacemaker pocket infection, the generator was removed and extraction of the electrodes was attempted without success. The patient follow-up was lost until November 2014, when he was admitted for several episodes of syncope and right failure. Tricuspid stenosis was diagnosed and surgery was chosen as treatment.

Keywords: stenosis; Pacemaker, artificial;

Introduction Case report

Tricuspid stenosis (TS), most of which is of rheumatic We report the case of a 72-year-old man who, in 1982, origin, is rarely seen in developed countries, although it in Switzerland, underwent surgical ablation for Wolf- is still found in developing countries.1 Other causes of Parkinson-White syndrome. The procedure was obstruction to right atrial emptying are unusual and complicated by complete atrioventricular block. include congenital , right atrial tumors, Permanent pacemaker was implanted. In 1992, in which can produce a clinical picture suggesting rapidly Portugal, the patient was hospitalized due to an infection progressive TS and .2 and necrosis of the pacemaker pocket and its leads. While in hospital, the generator was removed and there was Obstruction to right ventricular inflow is rarely caused an unsuccessful attempt to extract the leads of the by endomyocardial fibrosis, tricuspid valve vegetations, permanent pacemaker, which remained inside the right cardiac tumors or pacemaker electrodes. Interestingly, ventricle. Patient follow-up was lost after hospital there have been tricuspid stenosis reports without discharge. associated valve insufficiency, most of which are pacemaker lead-induced.3 In November 2014, the patient was admitted to the emergency room due to several episodes of syncope, We report a case of severe functional TS by intense edema of the lower limbs, decreased urine output and fibrosis and calcification of the pacemaker electrodes. ascites. Physical examination showed distension of the The patient was admitted to the hospital for right heart jugular veins, systolic and diastolic low frequency failure and atrioventricular block with indication of murmurs grade II/VI, hepatomegaly, ascites and permanent pacemaker. lower limb edema extending to the upper thigh.

Corresponding author: Ana Rita Pereira Alves Ferreira Rua Primeiro de Maio, 11 – Requião – 4770-454 – Vila Nova de Famalicão, Portugal E-mail: [email protected]

DOI: 10.5935/2359-4802.20150071 Manuscript received on July 15, 2015; approved on August 24, 2015; revised on October 14, 2015. 150 Ferreira et al. Int J Cardiovasc Sci. 2016;29(2):149-151 Tricuspid Stenosis Case Report

Electrocardiogram showed to the tricuspid valve, causing severe stenosis. Coronary ABBREVIATIONS AND ACRONYMS with angiography revealed no . some periods of complete • TS — tricuspid stenosis atrioventricular block. The patient was transferred to the cardiothoracic surgery • TV — tricuspid valve department, where the tricuspid valve was replaced by Chest radiography showed a biological prosthesis and an epicardial pacemaker system was implanted. During the surgery, a very and two calcified pacemaker leads dysmorphic TS was observed, with extremely small inside the right ventricle (Figure 1). Transthoracic opening and pronounced fibrosis and calcification over showed right atrial dilation, calcified the eletrocateter course. The remaining length of stay pacemaker leads adhering to the tricuspid valve (TV), was uneventful and five months after the surgery the producing severe functional stenosis (functional area patient was asymptomatic, and the bioprosthesis had 0.9 cm2 and average 6 mmHg gradient) and mild normal function. impairment without other significant abnormalities (Figure 2). Transesophageal echocardiography was performed to further characterize the TV morphology. Discussion This revealed two pacemaker leads in the right heart The most common form of TS is rheumatic and is usually chambers, one of which extremely calcified and adhering associated with other valvular diseases.1,2 This case illustrates a very rare form of TS, with only a few cases reported in the literature. The mechanisms described are right ventricular inflow obstruction by several pacemakers (in this case, by two electrodes) and fibrosis secondary to mechanical trauma associated with endocardial systems, perforation or laceration of the tricuspid leaflets, or adhesion between the redundant loops and valve tissue. The resulting endothelial damage triggers many local events, including chronic inflammation, fibrosis, calcification and, in some cases, valve stenosis, the latter being rarer. In most cases, TS was associated with more than one lead to cross the valve.3,4 The time between pacemaker implantation and early development of symptoms ranged from 7 to 33 years.5 In this case, the onset of symptoms occurred 22 years later. Figure 1 Chest radiograph showing cardiomegaly and two pacemaker leads inside the calcified right ventricle. There are some treatments described in the literature: some cases refer to medical treatment3, other cases refer

Figure 2 Transthoracic echocardiography showing right atrial dilation, calcified pacemaker leads adhering to the tricuspid valve, producing a severe functional stenosis. Int J Cardiovasc Sci. 2016;29(2):149-151 Ferreira et al. 151 Case Report Tricuspid Stenosis

to surgical replacement or surgical valve repair and, in should always be considered in patients with right heart fewer cases, balloon angioplasty. Percutaneous tricuspid failure and electrodes present in the right heart chambers. valvuloplasty may be a therapeutic alternative to surgery The therapeutic approach should be individualized, in cases of TS alone without any concomitant include a multidisciplinary team and the patient’s desires valvulopathy.4 In the case reported in this study, the must be considered. tricuspid valve was very calcified and dysmorphic and surgical treatment was chosen for the patient. Potential Conflicts of Interest This study has no relevant conflicts of interest. The TS induced by pacemaker leads is a chronic complication of pacemaker implantation that may occur Sources of Funding more often than it is clinically suspected and may become This study had no external funding sources. even more common due to the growing number of Academic Association cardiac devices currently implanted. The diagnosis This study is not associated with any graduate programs.

References

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