Case report Massive left atrial and interatrial septal

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Keywords: calcification; left atrial; interatrial septal; mitral valve replacement assive calcification of left ... prosthetic valve failure. Degeneration and perforation of.

Chinese Medical Journal 2008; 121(15):1497-1499

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Case report Massive left atrial and interatrial septal calcification after mitral valve replacement Yu-Shen Lin, Feng-Chun Tsai and Pao-Hsien Chu Keywords: calcification; left atrial; interatrial septal; mitral valve replacement

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assive calcification of left atrium is an uncommon complication of long-standing rheumatic valvular disease, and is most often observed in patients with a previous operation on mitral valve.1 Most patients have experienced symptoms for more than 15 years.2 Massive calcification of the left atrium generally spared the interatrial septum in the previous studies.2 However, to our knowledge, fewer than five cases have presented as full left atrial calcification (Table).1,3-5

dilated LA, LV, moderate mitral regurgitation (suspected perivalvular leakage), mild tricuspid regurgitation and adequate LV function. Additionally, spontaneous contrast over LV and LA has been observed in studies closed to third operation. Another cardiac catheterization was arranged in 2002 due to persistent moderate mitral regurgitation, showing an end-diastolic pressure gradient of 16 mmHg, grade 2/4 mitral regurgitation and mild pulmonary hypertension.

CASE REPORT

The patient was in NYHA class II–III suffering from intermittent leg edema, abdominal fullness and exertional dyspnea until he was 45 years old. Hemogram revealed progressed normocytic anemia (99 g/L, 88 g/L) and impressed hemolytic anemia. Further echocardiography showed progressed tricuspid regurgitation (moderate to severe), pressure gradient across the mitral valve (maximum pressure gradient: 40–50 mmHg) and moderate pulmonary hypertension (51 mmHg). He was admitted again for NYHA class III status and leg edema in September 2005, when he was 46 years old. On admission, eletrocardiography revealed atrial fibrillation with rapid ventricular response and left ventricular hypertrophy by voltage. Chest roentgenography revealed cardiomegaly and calcification of the LA (Figure 1). Moreover, the echocardiogram showed interatrial septal calcification (Figure 2). Catheterization demonstrated moderate pulmonary hypertension (pulmonary artery pressure: 43 mmHg), grade 2–3/4 mitral regurgitation (perivalvular leakage) and moderate tricuspid regurgitation, but adequate left ventricular systolic function and normal coronary arteries. An operation for decompensation of heart failure related to failure of mitral prosthetic valve was performed in 2005.

The subject of this case study was a 47-year-old male patient, who was admitted to our hospital for progressed exertional dyspnea when he was 22 years old. On that admission, several work-ups revealed left ventricular hypertrophy and atrial fibrillation with rapid ventricular response, dilated left atrium (LA) and left ventricle (LV). Catheterization then demonstrated a peak gradient across the mitral valve of 19 mmHg, moderate pulmonary hypertension (37 mmHg) and mild mitral regurgitation. The diagnosis was rheumatic heart disease with severe mitral stenosis and mild mitral regurgitation. Mitral valve replacement with 29# Edward Carpentier valve (Edwards Lifesciences, USA) was then performed. The condition returned to New York Heart Association (NYHA) function class I status after the operation. Seven years later, when he was 29 years old, he was admitted again for acute decompensation of heart failure with acute pulmonary edema. He returned to NYHA class II–III status under medication therapy. Echocardiography showed dilated LA and LV, velocity of 2.2 m/s across mitral valve and severe mitral regurgitation. Cardiac catheterization demonstrated high pulmonary wedge pressure (28 mmHg), end-diastolic pressure gradient cross mitral valve of 14 mmHg and moderate to severe pulmonary hypertension (53 mmHg). A mitral valve replacement operation was performed using a 31# Sorine valve (Sorin Biomedical, USA) under the suspicion of prosthetic valve failure. Degeneration and perforation of the prosthetic valve were discovered during surgery. He was in NYHA class I and atrial fibrillation with moderate ventricular response under medication of oral digoxin and furosemide for several years following surgery. Series follow-up echocardiography in clinic showed

The First Cardiovascular Division, Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, China (Lin YS and Chu PH) Division of Thoracic & Cardiovascular Surgery, Chang Gung Memorial Hospital; Chang Gung University College of Medicine, Taipei, Taiwan, China (Tsai FC) Correspondence to: Pao-Hsien Chu, The First Cardiovascular Division, Department of Internal Medicine, Chang Gung Memorial Hospital, 199 Tun-Hwa North Road, Taipei 105, Taiwan, China (Tel: 886-3-328-1200 ext 8162. Fax: 886-3-327-1192. Email: [email protected]) This study was supported to Dr. Pao-Hsien Chu by NHRI, Taiwan, China (No. NHRI-EX95-9108SC, NHRI-EX96-9627SI); and NSC Memorial Hospital, and NSC (No. 94-2314-B-182-071) and the National Sciences Council (No. 95-2314-B-182-021).

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Table. Characteristics of cases with massive atrial calcification, including inter-atrial septal calcification No. 1 2 3 4

Gender NA Male Female Male

Age (years) NA 56 67

RHD + + +

History of operation for MV disease Operation method + Trans-septal approach for MVR with total endoatriectomy – Trans-septal approach for MVR with total endoatriectomy mitral commissurotomy Trans-septal approach for MVR with total endoatriectomy (died in 48 hours after surgery) 61 + MVR Prosthetic reconstruction of the left atrium by a T shaped vascular graft with total endoatriectomy AF: atrial fibrillation; MV: mitral valve; MVR: mitral valve replacement; NA: not available; RHD: rheumatic heart disease.

Reference 1 3 4 5

heart status. He was in NYHA class II with grade I edema one year after the surgery. Follow-up echocardiography showed dilated LA and LV, and mitral stenosis (velocity: 3.6 m/s, max pressure gradient: 51 mmHg). However, no significant pulmonary hypertension, mitral regurgitation or tricuspid regurgitation was detected. DISCUSSION

Figure 1. The left lateral view of the chest roentgenography illustrating the calcification of the left atrial wall (arrowhead).

Figure 2. The echocardiogram demonstrating severe calcification of the interatrial septum (arrowhead). LA, left atrium; RA, right atrium.

Through a median resternostomy, significant cardiac adhesion to sternum owning to previous operation twice was noted, but no RV perforation appeared during sternotomy. Following cadiopulmonary bypass from aorto-bicaval cannulation and trans-septal approach for mitral surgery, posterior annulus (P2 and P3) of mitral valve dehiscence with LV wall with impending rupture was found, and was repaired with equine pericardium 4 cm × 2 cm (Edwards Lifesciences); significant annulus calcification extending to LA and LV was also noted, and extensive decalcification was performed. Mitral replacement was then performed with a St Jude 31# mechanical valve. Additionally, dilatation of annulus of tricuspid valve was observed, and annuloplasty with Carpentier-Edwards classic tricuspid annuloplasty ring 32# (Edwards Lifesciences) was conducted under beating

This investigation presents a case of chronic rheumatic heart disease and atrial fibrillation for 23 years and three opened mitral valve replacements. In the third admission for operation for dysfunction of prosthetic mitral valve, calcification over mitral annulus, free wall of left atrial and interatrial septum in follow-up echocardiography and incomplete circular radio-opaque lesion surrounding the left atrial area in lateral chest roentgenography were found. Massive calcification of the left atrium, including the interatrial septum, was confirmed in operation. Although, previous investigation studies concluded atrial calcification may relate to rheumatic endocarditis,2 excessive stretch of the atrial bands3 or operation for mitral valve disease,1,5 atrial calcification was uncommom and even only four cases combined with interatrial septal calcification were found. Characteristics of these four cases were listed in the table. In 1960, 1%–2 % of the rheumatic population developing atrial calcification was estimated and it also made mitral valvulotomies not to be complete.2 In presented cases so far, operation for mitral valve replacement in these patients may face three problems: (1) hemostatic closure of the atriotomy; (2) complex approach to the left atrium and mitral valve annulus, and (3) high risk of thrombus formation.1,5 According to the Vallejo and coworkers’ experiences, modified mitral valve replacement with approaching the left atrium through the right atrium and interatrial septum (Dobost`s incision) combined with endoatriectomy was favored in these cases with atrial calcification.1 A total left atrium resection with T shape vascular graft replacement may even be adopted in selected cases with heavy calcification.5 However, the left atrial calcification was found first in operation in some cases.2 Some cases were diagnosed by chest roentgenography, particularly lateral view before pre-operative evaluation, before the 1960s.4 The development of echocardiography in the last decade has shifted focus to the image of the left atrium calcification

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involved in cases with rheumatic heart disease and even with previous operation for mitral valve disease.1,6 Although left calcification prevents transesophageal or transthoracic cardioechography from delineating intracardiac anatomy,7,8 we can pay more attention to the possibility of the left atrial calcification and may predict risk of thrombus formation and modify the operative procedure in rheumatic heart disease planned to accept operation according to the study. However, there is no effective medical prevention of interatrial septal calcification until now. Therefore, we recommend taking care with the image with abnormal high echo-density in rheumatic heart disease before operation. Furthermore, as in the case presented in this investigation, rare cases involving calcification of the interatrial septum may be more complex when approaching the left atrium, which changes the surgical decision. The interatrial septum condition should also be addressed in patients with long-standing rheumatic heart disease and previous opened mitral valve replacement.

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Vallejo JL, Merino C, Gonzalez-Santos JM, Bastida E, Albertos J, Riesgo MJ, et al. Massive calcification of the left atrium: surgical implications. Ann Thorac Surg 1995; 60:

1226-1229. Harthorne JW, Seltzer RA, Austen WG. Left atrial calcification. Review of the literature and proposed management. Circulation 1966; 34: 198-210. Ruvolo G, Greco E, Speziale G, Mercogliano D, Marino B. “Mold-like” calcification of the left atrium and of the pulmonary veins. Total endoatriectomy in a patient undergoing mitral valve replacement. Eur J Cardiothorac Surg 1994; 8: 54-55. Del Campo C, Weinstein P, Kunnelis C, DisStefano P, Ebers GM. Coconut atrium: transmural calcification of the entire left atrium. Tex Heart Inst J 2000; 27: 49. Santini F, Pierfranco P, Mazzucco A. Mitral valve replacement associated with massive left atrial calcification. Ann Thorac Surg 1998; 65: 1465-1468. Vijayvergiya R, Jeevan H, Grover A. Left atrial calcification in rheumatic heart disease: a rare presentation. Heart 2006; 92: 1218. Vilacosta I, Gomez J, Almeria C, Castillo JA, San Roman JA, Zamorano J, et al. Massive left atrial calcification: a limitation of transesophageal echocardiography. Am Heart J 1994; 127: 461. Goel AK, Singh B. Transesophageal echocardiography in the presence of left atrial calcification. J Am Soc Echocardiogr 1997; 10: 677.

(Received December 25, 2007) Edited by WANG Mou-yue and LIU Huan

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