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Archives of Medical Science
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vol. 14
 
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abstract:
Letter to the Editor

Giant coronary sinus secondary to partial anomalous pulmonary venous connection with combined mitral valve disease

Błażej Michalski, Piotr Lipiec, Łukasz Chrzanowski, Jarosław D. Kasprzak

Arch Med Sci 2018; 14, 2: 463–465
Online publish date: 2016/05/20
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Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Nowadays, these patients are diagnosed when still in childhood and operated on with excellent results [1]. The drainage directly to the coronary sinus accounts for 3% of patients with PAPVC, and only a few such cases with coexisting mitral valve disease are described in the literature [2, 3]. Partial anomalous pulmonary vein connection most commonly presents with an atrial septal defect (ASD), reportedly in 80–90% of cases. Of these, 85% are reportedly sinus venosus type, while 10–15% are secundum type [4, 5].
A 55-year-old woman with mitral valve disease of rheumatic etiology, atrial fibrillation, and previous ischemic stroke was admitted to the department of cardiology due to shortness of breath, decreased exercise tolerance, and fatigue. The symptoms of cardiac failure had been exacerbating for 6 months. That was the first hospitalization of the patient due to exacerbation of the symptoms of heart failure. Previously, the patient had been treated in the outpatient clinic only with diuretics, beta blockers and oral anticoagulation therapy (OAT). Her heart failure therapy was intensified with -blocker, ACE inhibitor and diuretics, but she gradually deteriorated to NYHA class III. Due to atrial fibrillation (AF) the medical therapy included OAT which was ineffective (INR = 1.65). Upon physical examination, elevated jugular venous pressure (JVP), loud S1 tone and split S2 tone with a slight murmur at the left upper sternal border, swollen ankles and shortness of breath were present. In ECG AF with right heart bundle branch block was present. Transthoracic echocardiogram (TTE) revealed enlargement of the right ventricle (37 mm) and right atrium (50 mm), severe tricuspid valve regurgitation, combined degenerative mitral valve disease with moderate stenosis (planimetric area 1.0 cm2) and regurgitation (Figure 1 C). Pulmonary hypertension was diagnosed with systolic pulmonary artery pressure of 55 mm Hg and a pulmonary to systemic flow ratio of 1 : 2.1. Additionally, enlargement of the coronary sinus (38 × 15 mm in parasternal long-axis view) (Figures 1 A, B) was noted. On transesophageal echocardiography (TEE) a large thrombus protruding from the left atrial appendage (LAA) was found, as well as patent foramen ovale (PFO) with minute...


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