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Cleft in the Anterior and Posterior Leaflet of the Mitral Valve: A Rare Anomaly
Cleft in the Anterior and Posterior Leaflet of the Mitral Valve: A Rare Anomaly Robert W. McDonald, RCVT, RDCS, Gary Y. Ott, MD, and George A. Pantely, MD, Portland, Oregon
A rare entity that causes congenital mitral regurgitation is an isolated cleft mitral valve. The cleft in the mitral valve can be seen in either the anterior or posterior leaflet of the valve. We present a unique case of an individual with a history of congenital mitral regurgitation caused by a cleft in both the anterior and posterior leaflets of the mitral valve. (JAM Soc EcHOCARDIOGR 1994;7:422-4.)
Congenital mitral regurgitation (MR) in isolation is a rare anomaly; it has been associated with other heart diseases such as cardiomyopathies, connective tissue and metabolic disorders, Kawasaki syndrome, and the rare case of cleft mitral valve with intact septa. 1 Although the occurrence of congenital MR with intact atrial and ventricular septa is rare, the anatomic features and two-dimensional echocardiographic findings of this anomaly have been described. 2"6
From the Divisions of Adult Cardiology and Cardiopulmonary Surgery, the School of Medicine, Oregon Health Sciences University. Reprint requests: Robert W. McDonald, RCVT, RDCS, Echo Lab L 462, Oregon Health Sciences University, 3181 S.W. Sam Jackson Park Rd., Portland, OR 97201-3098. Copyright© 1994 by the American Society ofEchocardiography. 0894-7317/94$3.00 + 0 27/1/53988
This case report describes an individual with the diagnosis of congenital MR caused by a cleft in the anterior and posterior leaflets of the mitral valve. CASE REPORT 33-year-old man was first seen at the Oregon Health Sciences University (OHSU) in 1962, at age 4 years, with a systolic thrill palpable along the left sternal border and a grade 4 to 6 pansystolic harsh murmur heard loudest along the left sternal border but also throughout the precordium. The electrocardiogram was normal. A right-sided heart catheterization (Table 1) demonstrated mild pulmonary hypertension with moderate elevation of mean pulmonary artery wedge pressure caused in part by a large V wave. No left-to-right shunt was detected by 0 2 contents, Coomassie blue dye curve, and hydrogen electrode curve. A diagnosis of congenital MR was made. Because he was symptom free, he was followed up with yearly visits. At the age of 9 years he underwent another cardiac catheterization to assess the severity of MR (Table 1). This demonstrated mild pulmonary hypertension with normal
Table 1 Cardiac catheterization and echocardiographic data Condition
Catheterization pressures (mm Hg) Right atrium (mean) Right ventricle Pulmonary artery Pulmonary artery (mean) Pulmonary artery wedge (mean) Left ventricle Echocardiographic dimensions (mm) Left ventricle Left atrium
422
1986
1962
1967
Rest
Rest
Rest
5 28/5 25/15 22 18
3 30/2 28/9 16 11 120/9
6 32/5 32/13 22 13 105/17 62 46
1992 Exercise
Rest
38/17 30 16 105/11
2 26/3 26/12 17 8 104/6 72 56
Joumal of the American Society of Echocardiography
McDonald, Ott, and Pantely 423
Volume 7 Number 4
Figure 1 Parasternal short-axis image of anterior leaflet of mitral valve with large cleft (arruws). R V, Right ventricle; LV, left ventricle.
left ventricular diastolic pressure. Left ventriculography showed moderate to severe MR. He was symptom free and had follow-up visits to the age of 23 years without any cardiac symptoms. In 1986, at the age of 28 years, he was admitted to another hospital with increased exertional fatigue and had a grand mal seizure with tonic/ clonic movements of the upper extremity. Hospital workup included negative blood cultures, normal computed tomographic scan except for antral sinusitis, and an electroencephalogram that was within normal limits. On electrocardiography he demonstrated atrial fibrillation but shortly thereafter spontaneously converted to normal sinus rhythm. He was discharged with warfarin (Coumadin) and phenytoin (Dilantin) and referred to OHSU for follow-up. At that time his first echocardiogram was done. This showed a cleft in the anterior portion of the mitral valve with thickened leaflets (Figure 1) and severe MR. No evidence of a ventricular or atrial septal defect was found; the dimension of the left atrium was 46 mm and the left ventricular cavity was 62 mm. Four months later cardiac catheterization was carried out to evaluate intracardiac shunts and the possibility of valve surgery (Table 1). Again, this demonstrated the mild pulmonary hypertension that increased slightly with exercise. The coronary arteries were normal. Valve replacement was not recommended and he was discharged with warfarin.
In 1991, at the age of 32 years, he was seen at another hospital and was treated successfully for bacterial endocarditis (microaerophilic streptococci), which was thought to involve the mitral valve. In 1992 he was seen at OHSU at age 33 years with severe fatigue and dyspnea. Another echocardiogram was obtained and again demonstrated a cleft in the anterior portion of the mitral valve, but a posterior cleft of the mitral valve was noted as well (Figure 2). The left atrial dimension was 56 mm and the left ventricular cavitary dimension was 72 mm. Doppler techniques demonstrated severe MR. He underwent a fourth cardiac catheterization (Table 1), which demonstrated normal right- and left-sided heart pressures. Left ventriculography showed severe MR. Because of increasing symptoms and increasing left ventricular size, he was taken to surgery. A large cleft in the anterior mitral valve leaflet, as well as a small cleft in the posterior leaflet, was found. A St. Jude 33 mm mitral valve prosthesis was placed and the postoperative course was unremarkable.
DISCUSSION
Isolated cleft mitral valve with intact atrial and ventricular septum is a rare anomaly. Although it has been shown that a cleft in either the anterior or pos-
Journal of the American Society of Echocardiography July-August 1994
424 McDonald, Ott, and Pantely
Figure 2 Parasternal short-axis image of posterior leaflet of mitral valve with small cleft (arrows). R V, Right ventricle; LV, left ventricle.
terior leaflets of the mitral valve can occur, 1-6 clefts in both leaflets in the same patient have not been described previously, to our knowledge. When echocardiography was first performed, the anterior cleft of the mitral valve was seen; however, the smaller posterior cleft was not noted. A later echocardiographic study demonstrated the cleft in the posterior leaflet of the mitral valve. Both clefts were confirmed at surgery. 6 Although the cleft in the posterior leaflet was first noted after the episode of endocarditis, the appearance and location of the cleft noted at surgery and on pathologic examination indicated that it was congenital and not related to the endocarditis. Although isolated cleft of the mitral valve is a rare entity, a careful examination of both leaflets is warranted in an individual with a long history of MR.
REFERENCES l. Baylen BG, Waldhausen JA. Disease of the mitral valve. In:
2. 3. 4.
5. 6.
Adams FH, Emmanouilides GC, Reiemenschneider TA, eds. Moss' heart disease in infants, children and adolescents. Baltimore: Williams & Wilkins, 1989:647-63. Greene AC, Kotler MN, Mintz GS, Eshaphpour E, Segal BL. Isolated cleft mitral valve: rare case of mitral regurgitation. J Cardiovasc Ultrasonogr 1982;1:13-8. Segni ED, Edwards JE. Cleft anterior leaflet of the mitral valve with intact septa. Am J Cardiol 1983;51:919-26. Segni ED, Bass JL, Lucas RV, Einzig S. Isolated cleft mitral valve: a variety of congenital mitral regurgitation identified by 2-dimensional echocardiography. Am J Cardiol 1983;51: 927-31. Barth CW, Dibdin JD, Roberts WC. Mitral valve cleft without cardiac septal defect causing severe mitral regurgitation but allowing long survival. Am J Cardiol 1985;55:1229-31. Silverman NH. Pediatric echocardiography. Baltimore: Williams & Wilkins, 1993:375.
A rare entity that causes congenital mitral regurgitation is an isolated cleft mitral valve. The cleft in the mitral valve can be seen in either the anterior or posterior leaflet of the valve. We present a unique case of an individual with a history of congenital mitral regurgitation caused by a cleft in both the anterior and posterior leaflets of the mitral valve. (JAM Soc EcHOCARDIOGR 1994;7:422-4.)
Congenital mitral regurgitation (MR) in isolation is a rare anomaly; it has been associated with other heart diseases such as cardiomyopathies, connective tissue and metabolic disorders, Kawasaki syndrome, and the rare case of cleft mitral valve with intact septa. 1 Although the occurrence of congenital MR with intact atrial and ventricular septa is rare, the anatomic features and two-dimensional echocardiographic findings of this anomaly have been described. 2"6
From the Divisions of Adult Cardiology and Cardiopulmonary Surgery, the School of Medicine, Oregon Health Sciences University. Reprint requests: Robert W. McDonald, RCVT, RDCS, Echo Lab L 462, Oregon Health Sciences University, 3181 S.W. Sam Jackson Park Rd., Portland, OR 97201-3098. Copyright© 1994 by the American Society ofEchocardiography. 0894-7317/94$3.00 + 0 27/1/53988
This case report describes an individual with the diagnosis of congenital MR caused by a cleft in the anterior and posterior leaflets of the mitral valve. CASE REPORT 33-year-old man was first seen at the Oregon Health Sciences University (OHSU) in 1962, at age 4 years, with a systolic thrill palpable along the left sternal border and a grade 4 to 6 pansystolic harsh murmur heard loudest along the left sternal border but also throughout the precordium. The electrocardiogram was normal. A right-sided heart catheterization (Table 1) demonstrated mild pulmonary hypertension with moderate elevation of mean pulmonary artery wedge pressure caused in part by a large V wave. No left-to-right shunt was detected by 0 2 contents, Coomassie blue dye curve, and hydrogen electrode curve. A diagnosis of congenital MR was made. Because he was symptom free, he was followed up with yearly visits. At the age of 9 years he underwent another cardiac catheterization to assess the severity of MR (Table 1). This demonstrated mild pulmonary hypertension with normal
Table 1 Cardiac catheterization and echocardiographic data Condition
Catheterization pressures (mm Hg) Right atrium (mean) Right ventricle Pulmonary artery Pulmonary artery (mean) Pulmonary artery wedge (mean) Left ventricle Echocardiographic dimensions (mm) Left ventricle Left atrium
422
1986
1962
1967
Rest
Rest
Rest
5 28/5 25/15 22 18
3 30/2 28/9 16 11 120/9
6 32/5 32/13 22 13 105/17 62 46
1992 Exercise
Rest
38/17 30 16 105/11
2 26/3 26/12 17 8 104/6 72 56
Joumal of the American Society of Echocardiography
McDonald, Ott, and Pantely 423
Volume 7 Number 4
Figure 1 Parasternal short-axis image of anterior leaflet of mitral valve with large cleft (arruws). R V, Right ventricle; LV, left ventricle.
left ventricular diastolic pressure. Left ventriculography showed moderate to severe MR. He was symptom free and had follow-up visits to the age of 23 years without any cardiac symptoms. In 1986, at the age of 28 years, he was admitted to another hospital with increased exertional fatigue and had a grand mal seizure with tonic/ clonic movements of the upper extremity. Hospital workup included negative blood cultures, normal computed tomographic scan except for antral sinusitis, and an electroencephalogram that was within normal limits. On electrocardiography he demonstrated atrial fibrillation but shortly thereafter spontaneously converted to normal sinus rhythm. He was discharged with warfarin (Coumadin) and phenytoin (Dilantin) and referred to OHSU for follow-up. At that time his first echocardiogram was done. This showed a cleft in the anterior portion of the mitral valve with thickened leaflets (Figure 1) and severe MR. No evidence of a ventricular or atrial septal defect was found; the dimension of the left atrium was 46 mm and the left ventricular cavity was 62 mm. Four months later cardiac catheterization was carried out to evaluate intracardiac shunts and the possibility of valve surgery (Table 1). Again, this demonstrated the mild pulmonary hypertension that increased slightly with exercise. The coronary arteries were normal. Valve replacement was not recommended and he was discharged with warfarin.
In 1991, at the age of 32 years, he was seen at another hospital and was treated successfully for bacterial endocarditis (microaerophilic streptococci), which was thought to involve the mitral valve. In 1992 he was seen at OHSU at age 33 years with severe fatigue and dyspnea. Another echocardiogram was obtained and again demonstrated a cleft in the anterior portion of the mitral valve, but a posterior cleft of the mitral valve was noted as well (Figure 2). The left atrial dimension was 56 mm and the left ventricular cavitary dimension was 72 mm. Doppler techniques demonstrated severe MR. He underwent a fourth cardiac catheterization (Table 1), which demonstrated normal right- and left-sided heart pressures. Left ventriculography showed severe MR. Because of increasing symptoms and increasing left ventricular size, he was taken to surgery. A large cleft in the anterior mitral valve leaflet, as well as a small cleft in the posterior leaflet, was found. A St. Jude 33 mm mitral valve prosthesis was placed and the postoperative course was unremarkable.
DISCUSSION
Isolated cleft mitral valve with intact atrial and ventricular septum is a rare anomaly. Although it has been shown that a cleft in either the anterior or pos-
Journal of the American Society of Echocardiography July-August 1994
424 McDonald, Ott, and Pantely
Figure 2 Parasternal short-axis image of posterior leaflet of mitral valve with small cleft (arrows). R V, Right ventricle; LV, left ventricle.
terior leaflets of the mitral valve can occur, 1-6 clefts in both leaflets in the same patient have not been described previously, to our knowledge. When echocardiography was first performed, the anterior cleft of the mitral valve was seen; however, the smaller posterior cleft was not noted. A later echocardiographic study demonstrated the cleft in the posterior leaflet of the mitral valve. Both clefts were confirmed at surgery. 6 Although the cleft in the posterior leaflet was first noted after the episode of endocarditis, the appearance and location of the cleft noted at surgery and on pathologic examination indicated that it was congenital and not related to the endocarditis. Although isolated cleft of the mitral valve is a rare entity, a careful examination of both leaflets is warranted in an individual with a long history of MR.
REFERENCES l. Baylen BG, Waldhausen JA. Disease of the mitral valve. In:
2. 3. 4.
5. 6.
Adams FH, Emmanouilides GC, Reiemenschneider TA, eds. Moss' heart disease in infants, children and adolescents. Baltimore: Williams & Wilkins, 1989:647-63. Greene AC, Kotler MN, Mintz GS, Eshaphpour E, Segal BL. Isolated cleft mitral valve: rare case of mitral regurgitation. J Cardiovasc Ultrasonogr 1982;1:13-8. Segni ED, Edwards JE. Cleft anterior leaflet of the mitral valve with intact septa. Am J Cardiol 1983;51:919-26. Segni ED, Bass JL, Lucas RV, Einzig S. Isolated cleft mitral valve: a variety of congenital mitral regurgitation identified by 2-dimensional echocardiography. Am J Cardiol 1983;51: 927-31. Barth CW, Dibdin JD, Roberts WC. Mitral valve cleft without cardiac septal defect causing severe mitral regurgitation but allowing long survival. Am J Cardiol 1985;55:1229-31. Silverman NH. Pediatric echocardiography. Baltimore: Williams & Wilkins, 1993:375.