Isolated cleft mitral valve: Valve reconstruction techniques

Isolated Cleft Mitral Valve: Valve Reconstruction Techniques Patrick Perier, MD, and Bernd Clausnizer, M D Herz und Gef/iss Klinik, Bad Neustadt/Saale, Germany

Reports concerning an isolated cleft of the anterior mitral valve are rare. This congenital anomaly of the mitral valve is usually repaired by suturing the edges of the cleft. We report 4 cases of isolated anterior mitral cleft. The patients ranged in age from 13 to 41 years. The clinical symptoms were those typical of mitral insufficiency. In all 4 patients, preoperative echocardiography was able to establish the exact anatomic diagnosis. In 1 patient, the cleft was directly sutured, whereas, in the other 3 patients, a fibrous reaction of the edges of the cleft with a subsequent lack of valvular tissue made direct

suture technically impossible. Instead, the fibrous edges of the cleft were resected and the anterior leaflet of the mitral valve was reconstructed using an autologous pericardial patch pretreated with buffered glutaraldehyde. All 4 patients underwent annuloplasty together with placement of a Carpentier mitral ring. Postoperative echocardiograms have confirmed good results of the repair; 1 patient has a trivial insufficiency and 3 have a completely competent mitral valve.

A

cleft of the anterior leaflet of the mitral valve. The atrial and ventricular septa were intact in all 4 patients.

cleft of the anterior leaflet of the mitral valve is usually associated with a septal defect of the endocardial cushion type (an atrioventricular septal defect). A cleft of the anterior mitral valve as an isolated anomaly is a rare cause of congenital mitral valve insufficiency [1-7]. Suturing of the cleft has been the only conservative surgical treatment carried out in the cases reported [4-20]. Four patients with an isolated cleft of the anterior leaflet of the mitral valve were operated on at our institution. The anatomic conditions allowed direct suturing of the cleft in 1 patient, but, in the other 3, the anterior mitral valve was partially replaced with an autologous pericardial patch treated with glutaraldehyde. We report our experience in these 4 patients, with a particular emphasis on the surgical techniques used. M e t h o d s and Results Between July 1990 and March 1993, 4 patients underwent mitral valve repair for an isolated cleft of the anterior leaflet of the mitral valve. The patient characteristics are summarized in Table 1. There were 2 male and 2 female patients.

Preoperative Cardiac Catheterization Findings The pulmonary artery pressures were moderately increased in 3 patients (patients 1, 2, and 3). No oximetric evidence of an intracardiac shunt was detected in any patient. Left ventricular cineangiography revealed the presence of severe mitral regurgitation in all patients. No patient had a gooseneck deformity or a clearly visible Accepted for publication June 22, 1994. Address reprint requests to Dr Perier, Herz und Gef~issKlinik, Salzburger Leite 1, 97616 Bad Neustadt/Saale, Germany.

© 1995 by The Society of Thoracic Surgeons

(Ann Thorac Surg 1995;59:56-9)

Preoperative Echocardiographic Findings The 4 patients u n d e r w e n t complete M-mode, twodimensional and Doppler echocardiographic study using Vingmed CFM-750 ultrasound equipment (Vingmed, Horten, Norway) before and after operation. In each patient, standard parasternal long-axis and short-axis as well as apical views were obtained. The characteristics studied are summarized in Table 1. Left ventricular outflow tract velocity was measured with continuouswave Doppler ultrasound to look for a subaortic obstruction. The mitral valve insufficiency was found to be severe in all 4 patients. The parasternal short-axis view clearly demonstrated the cleft anterior mitral valve leaflet in the 4 patients. Huge annulus deformation and dilatation was recognized in the 4 patients as an associated mechanism responsible for the mitral valve insufficiency. No accessory chordae tendineae and no left ventricular outflow tract obstruction were observed, and no atrial or ventricular septal defect could be detected. In patient 2, echocardiography confirmed the presence of a moderate tricuspid insufficiency stemming from dilatation of the annulus.

Operative Findings and Surgical Techniques The cardiopulmonary bypass and aortic cross-clamping times were 97 _+ 7 and 63.2 -+ 7.4 minutes, respectively. In patient 1, the cleft was not at the level of the center of the leaflet, the posterior portion of the anterior leaflet being wider than the anterior portion. The cleft extended for 75% of the distance between the free margin and the base of the anterior mitral leaflet. The cleft was I-shaped. The junction between the edges of the cleft and the free 0003-4975/95/$9.50 0003-4975(94)00613-C

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PERIER AND CLAUSNIZER ISOLATED CLEFT MITRALVALVE

57

Table 1. Patient Characteristics Patient No. Characteristics Age (y) Heart rhythm QRS axis (degrees) NYHA functional class Preoperative echocardiographic findings LA (mm) LVEDD (mm) LVESD (mm) FS LVOT (m/s) Postoperative echocardiographic findings LA (ram) LVEDD (ram) LVESD (ram) FS LVOT (m/s) MV area (cm 2) MI

1

2

3

4

13 SR +90 II

26 AF +80 III

25 SR +45 II

41 SR +50 II

Mean + SD 26 _+ 5.74 ... ... ...

61 69 36 0.48 1.2

68 78 54.5 0.22 0.89

45 58 35 0.26 0.8

40 46 33 0.27 0.2

53 _+ 6.5 62 ± 6.8 39 _+ 4.9 0.31 + 0.06 0.8 ± 0.2

46 57 39 0.31 1.2 2.5 0

66 70 58 0.18 1.25 2.2 0

40 50 30 0.25 1.25 2.4 Trivial

33 39 29 0.26 1.6 4.1 0

46 -+ 7.1 54 ± 6.6 39 ~ 6.7 0.25 ± 0.03 1.3 ± 0.9 2.8 -+ 0.4

AF - atrial fibrillation; FS - fractional shortening; LA - left atrium diameter; LVEDD ~ left ventricular end-diastolic diameter; LVESD = left ventricular end-systolic diameter; LVOT left ventricular outflow tract velocity; MI - mitral valve insufficiency; MV area mitral valve area; SD = standard deviation; SR = sinus rhythm.

e d g e of t h e a n t e r i o r mitral leaflet was s h a r p a n d a s s u m e d a 9 0 - d e g r e e angle. T h e e d g e s of the cleft w e r e relatively thin a n d smooth. T h e entire l e n g t h of t h e cleft was s u t u r e d w i t h 5-0 m o n o f i l a m e n t i n t e r r u p t e d s u t u r e s (Teflex; L a b o r a t o i r e s Peters, Bobigny, France), i m p a r t i n g a n e a r - n o r m a l a p p e a r a n c e to the mitral v a l v e w i t h the e x c e p t i o n of dilatation of the a n n u l u s . A No. 32 C a r p e n t i e r - E d w a r d s mitral r i n g (Baxter H e a l t h c a r e , Santa Ana, CA) w a s t h e n i m p l a n t e d (Fig 1). In the 3 o t h e r patients, the cleft was s h a p e d like an i n v e r t e d V, w i t h the a p e x p o i n t i n g t o w a r d the b a s e of the a n t e r i o r mitral leaflet. The cleft e x t e n d e d for 50% to 70% of t h e d i s t a n c e b e t w e e n the free m a r g i n a n d the b a s e of t h e a n t e r i o r m i t r a l leaflet a n d was l o c a t e d in the m i d d l e of the a n t e r i o r leaflet. T h e j u n c t i o n b e t w e e n the m a r g i n s of the cleft a n d the free e d g e of the a n t e r i o r leaflet was s m o o t h a n d a s s u m e d a c o n t i n u o u s c u r v e d line. N o r m a l

Fig 2. Surgical steps involved in resecting the cleft area of the anterior leaflet and reconstructing this leaflet with an autologous pericardial patch pretreated with glutaraldehyde. c h o r d a e t e n d i n e a e e x t e n d e d f r o m the a n t e r i o r a n d p o s terior p a p i l l a r y m u s c l e s to the a n t e r i o r a n d p o s t e r i o r h a l v e s of t h e a n t e r i o r leaflet, respectively. N o a c c e s s o r y c h o r d a e t e n d i n e a e w e r e a t t a c h e d to the e d g e s of the cleft. The e d g e s of the cleft w e r e r o l l e d a n d t h i c k e n e d b y n o d u l a r fibrous tissue. This sclerotic p r o c e s s p l a y e d a m a j o r role in a c c e n t u a t i n g t h e w i d t h of the cleft a n d in t h e s u b s e q u e n t lack of v a l v u l a r tissue of t h e a n t e r i o r leaflet (Fig 2). G i v e n t h e large a m o u n t of v a l v u l a r tissue m i s s i n g , s i m p l e s u t u r i n g of t h e cleft was n o t feasible. A n autolog o u s p e r i c a r d i a l p a t c h w a s h a r v e s t e d a n d t r i m m e d to r e m o v e fat a n d p l e u r a l tissue. It was t h e n p l a c e d in a 0.62% g l u t a r a l d e h y d e - b u f f e r e d s o l u t i o n (Baxter H e a l t h care) at r o o m t e m p e r a t u r e for at least 15 m i n u t e s b e f o r e b e i n g rinsed. T h e fibrous tissue on b o t h sides of the cleft was t h e n resected. A p a t c h of this a u t o l o g o u s t r e a t e d p e r i c a r d i u m was f a s h i o n e d in a t r i a n g u l a r s h a p e a n d s u t u r e d to the a n t e r i o r leaflet w i t h a 5-0 m o n o f i l a m e n t c o n t i n u o u s s u t u r e to r e p a i r the gap in the a n t e r i o r leaflet. The a s s o c i a t e d dilatation of t h e a n n u l u s was t h e n corr e c t e d in all p a t i e n t s t h r o u g h the i m p l a n t a t i o n of a C a r p e n t i e r - E d w a r d s r i n g (Nos. 36, 34, a n d 32) (see Fig 2). In P a t i e n t 2, the right a t r i u m was o p e n e d to allow i n s p e c t i o n of the t r i c u s p i d valve. A n u n s u s p e c t e d p a t e n t f o r a m e n o v a l e w a s d i s c o v e r e d a n d closed w i t h a direct suture. The t r i c u s p i d a n n u l u s was f o u n d to be w i d e l y dilated a n d a No. 34 C a r p e n t i e r - E d w a r d s r i n g w a s i m planted.

Postoperative Echocardiography The 4 p a t i e n t s u n d e r w e n t e c h o c a r d i o g r a p h y b e f o r e discharge. In 3, the mitral v a l v e was n o t e d to be totally c o m p e t e n t , b u t trivial r e g u r g i t a t i o n was p r e s e n t in the fourth. The p o s t o p e r a t i v e e c h o c a r d i o g r a p h i c findings are g i v e n in T a b l e 1.

Follow-up The p a t i e n t s h a v e b e e n f o l l o w e d up for 42, 28, 19, a n d 16 m o n t h s after o p e r a t i o n . All 4 are in N e w York H e a r t A s s o c i a t i o n f u n c t i o n a l class I in sinus r h y t h m a n d n o n e r e q u i r e a n t i c o a g u l a t i o n or any cardiac m e d i c a t i o n . Foll o w - u p e c h o c a r d i o g r a p h y has s h o w n v e r y stable results, w i t h a totally c o m p e t e n t m i t r a l v a l v e in 3 p a t i e n t s a n d trivial r e g u r g i t a t i o n in 1.

Comment Fig 1. Surgical steps involved in directly suturing an isolated cleft of the mitral valve.

Isolated cleft of the mitral v a l v e is a rare c o n g e n i t a l m a l f o r m a t i o n that was first d e s c r i b e d in 1954 [21]. The

58

PERIERAND CLAUSNIZER ISOLATEDCLEFTMITRALVALVE

nosologic relationship b e t w e e n the isolated mitral cleft a n d the endocardial cushion defect remains controversial. Anatomic a n d echocardiographic studies have shown that some anatomic features are specific to the isolated mitral cleft [19, 22]: unlike the endocardial cushion defect, the mitral a n n u l u s is in a n o r m a l position, the cleft points toward the left ventricular outflow tract, a n d the mitral a n d tricuspid valves are attached to the s e p t u m at different levels, with the tricuspid valve attached more inferiorly. As pointed out b y A n d e r s o n a n d associates [23], approximating the two edges of the breach restores the n o r m a l a n a t o m y of the anterior leaflet of the mitral valve in the setting of an isolated cleft mitral valve. However, approximating the two segments of the divided anterior leaflet of the left valve in the setting of an atrioventricular septal defect does not produce a n o r m a l anterior mitral valve. Except in rare cases [24, 25], an isolated cleft mitral valve is usually associated with a mitral valve insufficiency that requires surgical intervention. It is now generally agreed that, w h e n feasible, mitral repair is preferable to mitral valve replacement [26, 27]. As reported here, direct suture of the cleft is not always technically feasible because of the lack of valvular tissue. In these difficult cases, techniques that extend the anterior leaflet make this feasible a n d the functional outcome of valve repair is probably improved, with a totally competent valve the e n d result. Glutaraldehyde-treated autologous pericardium has b e e n the preferred patch material in these operations. Results from experimental a n d clinical studies that have assessed the qualities of the material have proved its stability and reliability [28, 29]. It is of interest that a suture of the cleft was possible only in the youngest patient, aged 13 years. Di Segni and Edwards [22] have already observed that a relationship exists b e t w e e n the age of the patients a n d the thickness of the cleft edges. The role of echocardiography in the diagnosis of the defect should be emphasized, as it has the specific capacity to differentiate a cleft mitral valve from other causes of congenital mitral valve insufficiency [19, 24, 30]. In conclusion, an isolated cleft mitral valve is a rare cause of congenital mitral valve insufficiency. W h e n e v e r feasible, suturing of the cleft a n d eventually annuloplasty should be carried out. W h e n there is a lack of substance due to fibrous shrinkage of the valvular tissue, resection of the edges of the cleft together with partial replacement of the anterior leaflet using autologous glutaraldehydetreated pericardium accomplishes a valve reconstruction associated with a good functional result.

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BOOKS

General Thoracic Surgery, 4th edition Edited by Thomas W. Shields, MD Baltimore, Williams and Wilkins, 1994 1,816 pp, illustrated, $198.00

Reviewed by Benedict D. T. Daly, MD The fourth edition of General Thoracic Surgery replaces the third edition published in 1989. In many ways the fourth edition resembles earlier ones. The organization and general format are similar. The contributors include leaders in various aspects of the science and practice of the specialty. Doctor Shields remains the author or co-author of 20% of the chapters and is clearly the principal contributor and architect of this work. The fourth edition, however, has been completely revised, updated, and expanded. It comes in two volumes with 50% more pages, a significant increase in the n u m b e r of illustrations, a more comprehensive index, expanded coverage of the newer aspects of general thoracic surgery, and significantly greater attention to the technical aspects of both common and uncomm o n thoracic procedures. The references are current through 1993 and some are as recent as early 1994. The book is divided u n d e r four headings. Under the first heading is a new a n d single chapter that introduces the specialty of general thoracic surgery and its early history. Considered u n d e r the second heading are the lung and its contiguous structures, the pleura, diaphragm, and chest wall. There are 16 sections with 101 chapters u n d e r this heading. All of the chapters have b e e n revised and several completely rewritten and reillustrated. The chapter on the lymphatics of the lung has been expanded with greater attention to the anatomy of the lobar and hilar nodes and the current theories of the routes of lymphatic drainage of the lungs to the mediastinal lymph nodes. The sections on pulmonary and tracheal resections exhaustively and authoritatively detail and illustrate all of the common operations in this area. In the chapter on lobectomy each operative maneuver is detailed for each lobe. The description of the technique of p n e u m o n e c t o m y is directed primarily to the excision of the lung in cancer patients; the author's use of a diaphragmatic flap to close a pericardial defect is interesting but probably will find little general acceptance. In the section on tracheal pneumonectomy the old concept of a two-staged procedure on the left is replaced by a more appropriate one-stage operation using a combined median sternotomy and left thoracotomy. Finally, the techniques of unilateral and bilateral mediastinal lymph node dissections are well described. The importance of staging in the m a n a g e m e n t of malignant disease is emphasized. In the section © 1995 by The Society of Thoracic Surgeons

on lung cancer, the recent advances in molecular biology and immunology are detailed. The principles of chemotherapy and radiation therapy are reviewed as well as their application in the nonoperative a n d adjuvant settings. The section on the neoadjuvant approach is cautious but probably should be more enthusiastic in light of the more recent literature and my own experience. The physiology of the pleura and pleural space are detailed. The section on pleural tumors has been expanded to include the new concept of solitary fibrous tumors of the pleura rather than the older and inappropriate discussion of benign pleural mesotheliomas. Recent advances in treatment and resection of malignant pleural mesotheliomas are finely detailed. The section on transplantation has been completely rewritten and expanded, and a chapter on video-assisted thoracic surgery has been added. Discussion devoted to the esophagus occurs u n d e r the third heading in the b o o k and coverage of this organ has been greatly increased. The anatomy, physiology, and diagnosis of esophageal disease all are detailed in several chapters devoted to this difficult organ. Two chapters are devoted to trauma, eight to benign esophageal disease including inflammatory diseases with a detailed discussion of Chagas' disease, and five to malignant esophageal disease. The section on operative procedures in the m a n a g e m e n t of esophageal disease is up to date and authoritatively details and extensively illustrates each of the major approaches to esophageal resection and replacement in a step-by-step approach. Each of these chapters reflects the personal experience of the contributing authors and is full of detail not easily found otherwise. Consideration of the mediastinum falls within the final heading of the book. It has been updated including a new chapter on video-assisted thoracic surgery. The coverage is complete; however, more comprehensive coverage of mediastinal disease can be found in Dr Shields" book Mediastinal Surgery. It is to be hoped that this book will be incorporated into the text in the next edition. General Thoracic Surgery is an important work. It defines the field of general thoracic surgery in a comprehensive fashion and provides a foundation of knowledge for those working in the field. The in-depth descriptions of the technical aspects of operations are a must for all learning these procedures. Finally, the book provides a readily available, almost encyclopedic but easily readable resource for the practitioner, resident, and medical student confronted with the diversity and complexity of conditions encountered in general thoracic surgical practice.

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