- Email: [email protected]
Spontaneous rupture of a fenestrated aortic valve
Spontaneous rupture of a fenestrated aortic valve Surgical treatment Fenestration of the aortic valve is a common anatomic finding. Although spontaneous rupture of the semilunar cusps is infrequent, it leads to rapidly progressive heart failure and death. We present, to our knowledge, the first case of successful treatment by valve reconstruction. A 2 year follow-up demonstrates excellent clinical results. Sergio V. Moran, M . D . , Pablo Casanegra, M.D., Gustavo Maturana, M.D., and Juan Dubernet, M . D . , Santiago, Chile
X\.upture of the aortic valve is caused most frequently by bacterial endocarditis, syphilis, or trauma. 1 - 3 Five cases of spontaneous rupture of a fenestrated aortic valve have been reported in the literature. 1, 4 " 6 The rupture has been associated with hypertension, syphilitic aortitis, or strain, and the clinical course has been sudden onset of progressive heart failure resistant to all forms of medical treatment. The purpose of this article is to report the first case of spontaneous rupture of a fenestrated aortic valve successfully managed by surgical intervention. Case report F. A., a 31-year-old man, was admitted to the Catholic University Hospital in August, 1973, with a one-month history of progressive shortness of breath. He had no previous history of cardiovascular disease, and a physical examination performed 4 years earlier had disclosed no abnormalities. One month prior to admission, while at rest, the patient suddenly experienced a severe, crushing midsternal pain associated with faintness and sweating. This episode subsided after one hour. In the following days he noticed progressive limitation of his activity because of dyspnea. Examination at this time revealed a well-developed man with peripheral signs of free aortic regurgitation. The blood pressure was 120/40 mm. Hg. The pulse rate was 96 beats per minute in sinus rhythm, and the apical impulse was forceful and was located lateral to the midclavicular line in the fifth From the Hospital Universidad Catolica, Santiago, Chile. Received for publication Sept. 23, 1976. Accepted for publication Nov. 5, 1976. Address for reprints: Dr. Sergio V. Moran, Hospital Universidad Catolica, Marcoleta 347, Santiago 1, Chile.
716
intercostal space. A diastolic thrill was palpable over the left sternal border, and a Grade 2 systolic ejection murmur was present in the aortic area. A loud diastolic decrescendo murmur was heard over the entire anterior part of the chest, especially over the aortic area. This murmur was audible without applying the stethoscope or the ear to the chest. Its acoustic characteristics were unusual, resembling the call of the seagull. The electrocardiogram revealed left ventricular strain and the chest x-ray film demonstrated moderate cardiomegaly with left ventricular predominance. Cardiac catheterization indicated left heart failure and moderate pulmonary hypertension (Table I). Retrograde aortographic studies showed Grade 4/4 aortic regurgitation. With the diagnosis of aortic valve rupture, the patient was operated upon in September, 1973. The aortic valve was explored with the aid of cardiopulmonary bypass and anoxic cardiac arrest in normothermia. The commissure between the right and noncoronary cusps had become completely detached from the aortic wall and prolapsed into the left ventricle. Both leaflets had a large fenestration located adjacent to their insertion into the valvular annulus. The aortic valve was otherwise normal. The valve was repaired by reuniting both leaflets with interrupted 4-0 Ti-Cron* sutures over pericardial pledgets to form a new commissure. This new commissure was then attached to the aortic wall, and the valve was thereby restored to its normal anatomy (Fig. 1). The valve was tested for competence with saline, and no regurgitation was noticed. The patient was easily weaned from bypass, and the postoperative course was uncomplicated. Four months after the operation, the patient was readmitted for evaluation. He was found to be in Functional Class I (N.Y.H.A.), and cardiac catheterization revealed normal pressures (Table I). Two years after the operation, the patient is asymptomatic and working full time. *Davis & Geek, American Cyanamid Company, Pearl River, N. Y.
Volume 73 Number 5 May, 1977
Spontaneous rupture of aortic valve
717
Fig. 1. Both the right and noncoronary cusps had become detached at their point of insertion by rupturing along the commissure and prolapsing into the left ventricle ( A ) . Surgical repair consisted in reapproximation of both leaflets by direct suture ( B ) andfixationto the original commissural insertion in the aortic wall ( C ) .
Discussion Although fenestration of the semilunar cusps is a frequent anatomic finding,7 it is rarely associated with regurgitation,8 because the valvular defect is located above the line of closure near the free edge of the cusp. However, this defect may extend downward and cause valvular insufficiency, or these weakened leaflets may rupture, either spontaneously, as in the present case, or secondary to strain, hypertension, or bacterial endocarditis. The result is rapidly progressive heart failure and d e a t h
i . 3 , 4, 6, 9
The cause of fenestration is not certain, but most authors believe it is an acquired degenerative lesion.4, 10 It is more common in the adult than in the infant, and its frequency increases with age. Histologic examination has demonstrated in some cases mucinous degeneration of the protein structure of the leaflets.9 Rupture occurs in the point of maximal weakness, characteristically located at the outer margin of the cusp, where there is only a threadlike strand attaching the cusp to the aortic annulus. 4,5 The clinical features of the cases previously reported closely resemble those of our case, with sudden onset of chest pain, hypotension, and paroxysmal dyspnea. The rapidly progressive heart failure that ensues, resistant to medical treatment, is accompanied by the typical findings of free aortic regurgitation. Some authors have described a peculiar diastolic murmur similar to the call of the seagull. This murmur seems to be common in cases of aortic valve rupture and was present in our patient.10 The unfavorable prognosis of aortic valve rupture is
Table I. Comparison of preoperative and postoperative catheterization data
Site Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Aorta Maximum dp/dt (mm. Hg/sec.) Cardiac index (L./min •)
Preop. pressure (mm. Hg) 3 36/3 36/15 22 96/38 92/41 600 3.0
Post op. pressure (mm. Hg) 5 23/5 23/9 7 115/8 110/70 1.760 4.2
well known. Early recognition and prompt surgical management are mandatory. In this particular case, the ruptured valve was reparable without the need for prosthetic replacement. The favorable postoperative course and the findings at the control catheterization study support this conduct. Because of the small but definite number of complications associated with aortic valve replacement,11, 12 valvuloplasty should be considered the treatment of choice.
REFERENCES 1 Carrol, D.: Nontraumatic Aortic Valve Rupture, Bull. Johns Hopkins Hosp. 89: 309, 1951. 2 Levine, R. J., Roberts, W. C , and Morrow, A. G.: Traumatic Aortic Regurgitation, Am. J. Cardiol. 10: 752, 1962.
7 18
The Journal of Thoracic and Cardiovascular Surgery
Mordn et al.
3 Najafi, H., Dye, W. S., Javid, H., Hunter, J. A., and Julian, O. C : Rupture of an Otherwise Normal Aortic Valve: Report of Two Cases and Review of the Literature, J. THORAC. CARDIOVASC. SURG. 56: 57,
1968.
4 Mathews, R. J., and Darville, F. T., Jr.: Fenestration of the Aortic Valve Cusps as a Cause of Aortic Insufficiency and Spontaneous Aortic Valve Cusp Rupture, Ann. Intern. Med. 44: 993, 1956. 5 Proudfit, W. L., and Mc Cormack, L. J.: Clinical Conference: Rupture of the Aortic Valve, Circulation 13: 750, 1956. 6 Marcus, F. I., Ronan, J., Misanik, L. F., and Gordon, A. E.: Aortic Insufficiency Secondary to Spontaneous Rupture of a Fenestrated Leaflet, Am. Heart J. 66: 675, 1963. 7 Foxe, A.: Fenestration and Semilunar Valves, Am. J. Pathol. 5: 179, 1929. 8 Friedman, B., and Hathaway, B.: Fenestration of the
Semilunar Cusps and Functional Aortic and Pulmonary Valve Insufficiency, Am. J. Med. 24: 549, 1958. 9 Castelman, B., and Towne, V. W.: Case Records of the Massachusetts General Hospital, N. Engl. J. Med. 245: 941, 1951. 10 Friedberg, C. K.: Diseases of the Heart, Philadelphia, 1966, W. B. Saunders Company, p. 1111. 11 Barnhorst, D. A., Oxman, H. A., Connolly, D. C , Pluth, J. R., Danielson, G. K., Wallace, R. B., and McGoon, D. C : Isolated Replacement of the Aortic Valve With Starr-Edwards Prosthesis: A 9 Year Review, J. THORAC. CARDIOVASC. SURG. 70: 113,
1975.
12 Winter, T. Q., Reis, R. L., Glancy, D. L., Roberts, W. C , Epstein, S. E., and MQITOW, A. G.: Current Status of the Starr-Edwards Cloth-Covered Prosthetic Cardiac Valves, Circulation 45, 46: 1, 1972 (Suppl. I).
X\.upture of the aortic valve is caused most frequently by bacterial endocarditis, syphilis, or trauma. 1 - 3 Five cases of spontaneous rupture of a fenestrated aortic valve have been reported in the literature. 1, 4 " 6 The rupture has been associated with hypertension, syphilitic aortitis, or strain, and the clinical course has been sudden onset of progressive heart failure resistant to all forms of medical treatment. The purpose of this article is to report the first case of spontaneous rupture of a fenestrated aortic valve successfully managed by surgical intervention. Case report F. A., a 31-year-old man, was admitted to the Catholic University Hospital in August, 1973, with a one-month history of progressive shortness of breath. He had no previous history of cardiovascular disease, and a physical examination performed 4 years earlier had disclosed no abnormalities. One month prior to admission, while at rest, the patient suddenly experienced a severe, crushing midsternal pain associated with faintness and sweating. This episode subsided after one hour. In the following days he noticed progressive limitation of his activity because of dyspnea. Examination at this time revealed a well-developed man with peripheral signs of free aortic regurgitation. The blood pressure was 120/40 mm. Hg. The pulse rate was 96 beats per minute in sinus rhythm, and the apical impulse was forceful and was located lateral to the midclavicular line in the fifth From the Hospital Universidad Catolica, Santiago, Chile. Received for publication Sept. 23, 1976. Accepted for publication Nov. 5, 1976. Address for reprints: Dr. Sergio V. Moran, Hospital Universidad Catolica, Marcoleta 347, Santiago 1, Chile.
716
intercostal space. A diastolic thrill was palpable over the left sternal border, and a Grade 2 systolic ejection murmur was present in the aortic area. A loud diastolic decrescendo murmur was heard over the entire anterior part of the chest, especially over the aortic area. This murmur was audible without applying the stethoscope or the ear to the chest. Its acoustic characteristics were unusual, resembling the call of the seagull. The electrocardiogram revealed left ventricular strain and the chest x-ray film demonstrated moderate cardiomegaly with left ventricular predominance. Cardiac catheterization indicated left heart failure and moderate pulmonary hypertension (Table I). Retrograde aortographic studies showed Grade 4/4 aortic regurgitation. With the diagnosis of aortic valve rupture, the patient was operated upon in September, 1973. The aortic valve was explored with the aid of cardiopulmonary bypass and anoxic cardiac arrest in normothermia. The commissure between the right and noncoronary cusps had become completely detached from the aortic wall and prolapsed into the left ventricle. Both leaflets had a large fenestration located adjacent to their insertion into the valvular annulus. The aortic valve was otherwise normal. The valve was repaired by reuniting both leaflets with interrupted 4-0 Ti-Cron* sutures over pericardial pledgets to form a new commissure. This new commissure was then attached to the aortic wall, and the valve was thereby restored to its normal anatomy (Fig. 1). The valve was tested for competence with saline, and no regurgitation was noticed. The patient was easily weaned from bypass, and the postoperative course was uncomplicated. Four months after the operation, the patient was readmitted for evaluation. He was found to be in Functional Class I (N.Y.H.A.), and cardiac catheterization revealed normal pressures (Table I). Two years after the operation, the patient is asymptomatic and working full time. *Davis & Geek, American Cyanamid Company, Pearl River, N. Y.
Volume 73 Number 5 May, 1977
Spontaneous rupture of aortic valve
717
Fig. 1. Both the right and noncoronary cusps had become detached at their point of insertion by rupturing along the commissure and prolapsing into the left ventricle ( A ) . Surgical repair consisted in reapproximation of both leaflets by direct suture ( B ) andfixationto the original commissural insertion in the aortic wall ( C ) .
Discussion Although fenestration of the semilunar cusps is a frequent anatomic finding,7 it is rarely associated with regurgitation,8 because the valvular defect is located above the line of closure near the free edge of the cusp. However, this defect may extend downward and cause valvular insufficiency, or these weakened leaflets may rupture, either spontaneously, as in the present case, or secondary to strain, hypertension, or bacterial endocarditis. The result is rapidly progressive heart failure and d e a t h
i . 3 , 4, 6, 9
The cause of fenestration is not certain, but most authors believe it is an acquired degenerative lesion.4, 10 It is more common in the adult than in the infant, and its frequency increases with age. Histologic examination has demonstrated in some cases mucinous degeneration of the protein structure of the leaflets.9 Rupture occurs in the point of maximal weakness, characteristically located at the outer margin of the cusp, where there is only a threadlike strand attaching the cusp to the aortic annulus. 4,5 The clinical features of the cases previously reported closely resemble those of our case, with sudden onset of chest pain, hypotension, and paroxysmal dyspnea. The rapidly progressive heart failure that ensues, resistant to medical treatment, is accompanied by the typical findings of free aortic regurgitation. Some authors have described a peculiar diastolic murmur similar to the call of the seagull. This murmur seems to be common in cases of aortic valve rupture and was present in our patient.10 The unfavorable prognosis of aortic valve rupture is
Table I. Comparison of preoperative and postoperative catheterization data
Site Right atrium Right ventricle Pulmonary artery Left atrium Left ventricle Aorta Maximum dp/dt (mm. Hg/sec.) Cardiac index (L./min •)
Preop. pressure (mm. Hg) 3 36/3 36/15 22 96/38 92/41 600 3.0
Post op. pressure (mm. Hg) 5 23/5 23/9 7 115/8 110/70 1.760 4.2
well known. Early recognition and prompt surgical management are mandatory. In this particular case, the ruptured valve was reparable without the need for prosthetic replacement. The favorable postoperative course and the findings at the control catheterization study support this conduct. Because of the small but definite number of complications associated with aortic valve replacement,11, 12 valvuloplasty should be considered the treatment of choice.
REFERENCES 1 Carrol, D.: Nontraumatic Aortic Valve Rupture, Bull. Johns Hopkins Hosp. 89: 309, 1951. 2 Levine, R. J., Roberts, W. C , and Morrow, A. G.: Traumatic Aortic Regurgitation, Am. J. Cardiol. 10: 752, 1962.
7 18
The Journal of Thoracic and Cardiovascular Surgery
Mordn et al.
3 Najafi, H., Dye, W. S., Javid, H., Hunter, J. A., and Julian, O. C : Rupture of an Otherwise Normal Aortic Valve: Report of Two Cases and Review of the Literature, J. THORAC. CARDIOVASC. SURG. 56: 57,
1968.
4 Mathews, R. J., and Darville, F. T., Jr.: Fenestration of the Aortic Valve Cusps as a Cause of Aortic Insufficiency and Spontaneous Aortic Valve Cusp Rupture, Ann. Intern. Med. 44: 993, 1956. 5 Proudfit, W. L., and Mc Cormack, L. J.: Clinical Conference: Rupture of the Aortic Valve, Circulation 13: 750, 1956. 6 Marcus, F. I., Ronan, J., Misanik, L. F., and Gordon, A. E.: Aortic Insufficiency Secondary to Spontaneous Rupture of a Fenestrated Leaflet, Am. Heart J. 66: 675, 1963. 7 Foxe, A.: Fenestration and Semilunar Valves, Am. J. Pathol. 5: 179, 1929. 8 Friedman, B., and Hathaway, B.: Fenestration of the
Semilunar Cusps and Functional Aortic and Pulmonary Valve Insufficiency, Am. J. Med. 24: 549, 1958. 9 Castelman, B., and Towne, V. W.: Case Records of the Massachusetts General Hospital, N. Engl. J. Med. 245: 941, 1951. 10 Friedberg, C. K.: Diseases of the Heart, Philadelphia, 1966, W. B. Saunders Company, p. 1111. 11 Barnhorst, D. A., Oxman, H. A., Connolly, D. C , Pluth, J. R., Danielson, G. K., Wallace, R. B., and McGoon, D. C : Isolated Replacement of the Aortic Valve With Starr-Edwards Prosthesis: A 9 Year Review, J. THORAC. CARDIOVASC. SURG. 70: 113,
1975.
12 Winter, T. Q., Reis, R. L., Glancy, D. L., Roberts, W. C , Epstein, S. E., and MQITOW, A. G.: Current Status of the Starr-Edwards Cloth-Covered Prosthetic Cardiac Valves, Circulation 45, 46: 1, 1972 (Suppl. I).