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Coexistent Mitral Valve Prolapse and Mitral Stenosis
Coexistent Mitral Valve Prolapse and Mitral Stenosis
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To the Editor: As Beasley and Kerber! mentioned in their recent article, the coexistence of mitral prolapse and mitral stenosis is uncommon. Therefore, I wish to report another case with special emphasis on its interesting auscultatory findings and characteristic operative appearances of the affected mitral valve. CASE REPORT
A 37-year-old woman had progressive exertional dyspnea for two years associated with a mitral regurgitant murmur following acute rheumatic fever at age of ten . Cardiac examination revealed an accentuated first sound followed bv a loud pansystolic apical murmur with a midsystolic click , and a snappy " th ird" heart sound which occurred 0.12 sec after the second sound, followed by a long mid-diastolic murmur. Echocardiogram (Fig 1) showed mitral valve prolapse, as well as thickened mitral leaflets with parallel but slow posterior motion during diastole. Simultaneous phonocardiogram revealed an accentuated first sound, a midsystolic click, a pansystolic murmur and a long diastolic murmur preceded by an earl y diastolic sound which occurred 0.12 sec aft'll" the second sound (Fig 1). This early diastolic sound occurs after the max imal opening of the mitral valve on the simultaneously recorded echocardiogram (Fig 1). Cardiac catheterization revealed mild pulmonary hypertension (40/20 mm Hg) and a mean mitral diastolic gradient of 4 mm Hg , Left ventriculography revealed systolic prolapse of a thickened mitral valve with marked mitral regurgitation. At operation, both leaflets oE the mitral valve, especially the anterior, prolapsed into the left at rium (Fig 2) . There was also significant mitral stenosis, as evidenced by fused commissures, shortened and thickened chordae and rolled edges (Fig 2) . The mitral valve was excised and replaced by a Shiley porcine heterograft. The patient remained asymptomatic postoperatively.
FIGURE 1. Ecbocardiogram showing both systolic mitral valve prolapse (arrows) and diastolic mitral valve stenosis with accentuated first sound (Sd, midsystolic click (x ), systolic murmur (sm ), diastolic click (arrowheads) and diastolic murmur (dm) on phonocardiogram. Note that the diastolic click occurs after the maximal opening of the mitral valve. maximal mitral opening when the redundant leaflets continue to balloon further into the left ventricle during diastole (Fig 1) . Thus, the concomitant presence of systolic mitral valve prolapse and diastolic mitral valve stenosis in the same patient makes echocardiographic examination 'an ideal and accurate noninvasive means for its diagnosis.
Tsung O. Cheng, M.D., F.C.C.P. The George Washington University Medical Center, Washington , D.C. Reprint requests: Dr. Cheng, 2150 Pennsylvania Avenue, NW, Washington, DC 20037
COMMENT
Combined mitral prolapse and mitral stenosis is uncommon . Only eight such cases have been reported in the Iiterature. l - a This rare concurrence is even more unusual when one considers the fact that both mitral valve prolapse and mitral stenosis are relatively common entities by themselves and rheumatic fever may often be the etiology in both. All the reported cases presented clinically as mitral stenosis, and the associated mitral prolapse was not suspected until echocardiography was performed to substantiate the diagnosis of mitral stenosis. The case reported here is the only one which manifested clinically as mitral regurgitation secondary to mitral prolapse, but had certain features suggestive of coexisting mitral stenosis, including an accentuated first sou nd, a mid-diastolic rumbling murmur and a snappy early diastolic sound. The early diastolic sound in this patient, which occurred too late for an opening snap due to mitral stenosis, is the diastolic click due to mitral valve prolapse (Fig 1). Although the typical auscultatory finding in mitral valve prolapse is a mid systolic click with or without a late systolic murmur;' a diastolic click may occasionally be present." The latter may be confused clinically with an opening snap. However, the echocardiogram offers a clear distinction, as opening snap coincides with cessation of the rapid motion of the diastolic opening of the mitral valve, but the diastolic click in mitral prolapse occurs after the
526 COMMUNICATIONS TO THE EDITOR
FIGURE 2. Operative views of combined prolapse (upper) and stenosis ( lower) of the mitral valve.
CHEST, 81: 4, APRil, 1982
1 Beasley B, Kerber K. Does mitral prolapse occur in mitral stenosis? Echocardiographic-angiographic observatioDS. Chest 1981; 80:56-60 2 Howard PG, Benchimol A, Desser KB. Prolapse of the atrioventricular valves and associated mitral stenosis. A case report. Arizona Med 1976; 33:789-92 3 Weinrauch LA, McDonald DG, DeSilva R. Mitral valve prolapse in rheumatic mitral stenosis. Chest 1977; 72: 752-56 4 Cheng TO: The click-murmur syndrome. A medical pendulum and a unifying concept. Chest 1978; 70:569-72 5 Cheng TO: Diastolic click in mitral valve prolapse. Arch Intern Med 1977; 137:696
A Clue to Pulmonary Embolism Obtained during Swan-Ganz Catheterization To the Editor:
An unusual clue to pulmonary embolism was noted in our laboratory during a recent right heart catheterization. CASE REPoRT
A 54-year-old woman was admitted with edema of the right arm and dyspnea. Evaluation revealed right subclavian vein thrombosis, malignant left pleural effusion, and a large pericardial effusion. Significant past history included repair of an atrial septal defect eight years previously. Pulmonary hypertension was present at that time. Right heart catheterization and pericardiocentesis was performed to determine the hemodynamic significance of the effusion and to obtain specimens for cytology. Under fluoroscopy, a Swan-Ganz catheter was advanced without difliculty via the right femoral vein to the right pulmonary artery. The pressure recordings were diagnostic of cardiac tamponade (elevated right heart diastolic pressures with equalization of right atrial, right ventricular diastolic, and intrapericardial preswoe; all 20 DUD Hg). The cardiac .output (thermodilu.ion) was dhninished (3.2 Llmin). We were not able to obtain pulmonary capillary wedge pressure. Sanguinous pericardJal fluid (1365 ml) was removed with dramatic improvement of the hemodynamic parameters. Within minutes the patient began to experience increasing respiratory difficulty with severe hypoxia unresponsive to mechanical ventilation. Attempts to repeat the measurement of cardiac output by thermodilution after pericardiocentesis were unsuccessful, despite performing the routine tests for catheter and computer malfunction and changing both the Swan-Ganz catheter and the cardiac output computer. Pulmonary angiography confirmed the presence of a massive embolus to the right main pulmonary artery. The patient was immediately placed on femoral-femoral cardiopulmonary bypass in the catheterization laboratory and taken immediately to surgery for emergency pulmonary embolectomy. Carcinomatosis involving the entire right hemothorax, including the pericardial surface, was documented.
=
The inability to measure cardiac output by thermodilution during Swan-Ganz catheterization may serve as a clue to pulmonary embolism if the usual causes of malfunction have been considered and excluded. Undoubtedly, the thermistor tip was lodged in the region of the clot, preventing accurate measurement of the temperature changes occurring during iced-water injection. Indeed, the SwanGanz tip was located in the same pulmonary artery as the
CHEST, 81: 4, APRIL, 1982
massive pulmonary embolus. One should consider pulmonary embolus in similar situations. ]tmnBt F. LewIs, M.D.; Timothy W. Anderson, M.D.; waUam H. Fennell, M.D.; and]tJmB8 B. Young, M.D., F.C.CP., Ben Toob General Hoapitlll, Houston
Reprint requests: D,. Young, 1200 Mouf'BUnd. 512D, Hous-
ton 77030
A Complication with Thermodilution Cardiac Outputs in Centrally-Placed Pulmonary Artery Catheters To the Editor:
The use of side-arm percutaneous central venous pressure introducer sets for the insertion of balloon-tipped pulmonary artery catheters is becoming increasingly popular. In addition to expediting the subclavian or intemal jugular insertion of the pulmonary artery catheter, they have the added feature of a side-arm fluid administration port. Recently, we performed a thermodilution cardiac.output study utilizing a 4-lumen Edwards thermodilution SwanGanz catheter inserted through a USCI "hemaquet" arterial/venous percutaneous catheter introducer sheath. Using the Edwards 9520A computer and 10 ml of iced injectate, cardiac output values of 24 L/min and 22 L/min were obtained. During the second injection, a retrograde surge of blood-tinged intravenous fluid was observed in the line infusing the side-port of the introducer sheath. Closing off the stopcock on this line before the next thermodilution injection resulted in cardiac outputs in the range of 9 L/min. These values were 'more consistent with the patient's clinical picture. The in vivo length of the pulmonary artery catheter measured at the hub of the introducer sheath was 42 CDl. Since the proximal line exit port is at 30 em, and the length of the introducer sheath and hub is 15 em, we concluded that the proximal port was still well within the lumen of the sheath. During injection of the 10 ml iced bolus, a significant portion of this volume was being forced retrograde into the side-arm intravenous line. As a result, a smaller volume of the injectate was actually being added to the venous blood, thus causing erroneously high cardiac output values. Closing off the stopcock to the side-arm intravenous line corrected this problem. Since pulmonary artery catheters placed either through the subclavian or internal jugular veins tend to have short in vivo catheter lengths, their placement within an introducer sheath increases the likelihood of the above problem occurring. Therefore, when performing thermodilution cardiac output studies through a pulmonary artery catheter utilizing an introducer sheath with a side-arm intravenous line, care must be taken to insure that the proximal port is beyond the tip of the sheath. Otherwise, fluid infusion through the side-arm should be from an infusion pump or the side-arm intravenous line should be temporarily turned off. Mark G. Bearss, B.S., R.R.T.; David N. ¥onutaB, M.S., R.R.T.; and William T. Allen, M.D., rac.r; Department of Cardiopulmonary Services, St. Joseph MerCfl H08fJital, Ann Arbor, Michigan
COMMUNICADONS TO THE EDITOR 527
''='!:. ~-
---
- ~m ~: ~
x, '
I
,/
.------;~.,.,
r
T
-
,,--..,.
~
·s m
-dm
To the Editor: As Beasley and Kerber! mentioned in their recent article, the coexistence of mitral prolapse and mitral stenosis is uncommon. Therefore, I wish to report another case with special emphasis on its interesting auscultatory findings and characteristic operative appearances of the affected mitral valve. CASE REPORT
A 37-year-old woman had progressive exertional dyspnea for two years associated with a mitral regurgitant murmur following acute rheumatic fever at age of ten . Cardiac examination revealed an accentuated first sound followed bv a loud pansystolic apical murmur with a midsystolic click , and a snappy " th ird" heart sound which occurred 0.12 sec after the second sound, followed by a long mid-diastolic murmur. Echocardiogram (Fig 1) showed mitral valve prolapse, as well as thickened mitral leaflets with parallel but slow posterior motion during diastole. Simultaneous phonocardiogram revealed an accentuated first sound, a midsystolic click, a pansystolic murmur and a long diastolic murmur preceded by an earl y diastolic sound which occurred 0.12 sec aft'll" the second sound (Fig 1). This early diastolic sound occurs after the max imal opening of the mitral valve on the simultaneously recorded echocardiogram (Fig 1). Cardiac catheterization revealed mild pulmonary hypertension (40/20 mm Hg) and a mean mitral diastolic gradient of 4 mm Hg , Left ventriculography revealed systolic prolapse of a thickened mitral valve with marked mitral regurgitation. At operation, both leaflets oE the mitral valve, especially the anterior, prolapsed into the left at rium (Fig 2) . There was also significant mitral stenosis, as evidenced by fused commissures, shortened and thickened chordae and rolled edges (Fig 2) . The mitral valve was excised and replaced by a Shiley porcine heterograft. The patient remained asymptomatic postoperatively.
FIGURE 1. Ecbocardiogram showing both systolic mitral valve prolapse (arrows) and diastolic mitral valve stenosis with accentuated first sound (Sd, midsystolic click (x ), systolic murmur (sm ), diastolic click (arrowheads) and diastolic murmur (dm) on phonocardiogram. Note that the diastolic click occurs after the maximal opening of the mitral valve. maximal mitral opening when the redundant leaflets continue to balloon further into the left ventricle during diastole (Fig 1) . Thus, the concomitant presence of systolic mitral valve prolapse and diastolic mitral valve stenosis in the same patient makes echocardiographic examination 'an ideal and accurate noninvasive means for its diagnosis.
Tsung O. Cheng, M.D., F.C.C.P. The George Washington University Medical Center, Washington , D.C. Reprint requests: Dr. Cheng, 2150 Pennsylvania Avenue, NW, Washington, DC 20037
COMMENT
Combined mitral prolapse and mitral stenosis is uncommon . Only eight such cases have been reported in the Iiterature. l - a This rare concurrence is even more unusual when one considers the fact that both mitral valve prolapse and mitral stenosis are relatively common entities by themselves and rheumatic fever may often be the etiology in both. All the reported cases presented clinically as mitral stenosis, and the associated mitral prolapse was not suspected until echocardiography was performed to substantiate the diagnosis of mitral stenosis. The case reported here is the only one which manifested clinically as mitral regurgitation secondary to mitral prolapse, but had certain features suggestive of coexisting mitral stenosis, including an accentuated first sou nd, a mid-diastolic rumbling murmur and a snappy early diastolic sound. The early diastolic sound in this patient, which occurred too late for an opening snap due to mitral stenosis, is the diastolic click due to mitral valve prolapse (Fig 1). Although the typical auscultatory finding in mitral valve prolapse is a mid systolic click with or without a late systolic murmur;' a diastolic click may occasionally be present." The latter may be confused clinically with an opening snap. However, the echocardiogram offers a clear distinction, as opening snap coincides with cessation of the rapid motion of the diastolic opening of the mitral valve, but the diastolic click in mitral prolapse occurs after the
526 COMMUNICATIONS TO THE EDITOR
FIGURE 2. Operative views of combined prolapse (upper) and stenosis ( lower) of the mitral valve.
CHEST, 81: 4, APRil, 1982
1 Beasley B, Kerber K. Does mitral prolapse occur in mitral stenosis? Echocardiographic-angiographic observatioDS. Chest 1981; 80:56-60 2 Howard PG, Benchimol A, Desser KB. Prolapse of the atrioventricular valves and associated mitral stenosis. A case report. Arizona Med 1976; 33:789-92 3 Weinrauch LA, McDonald DG, DeSilva R. Mitral valve prolapse in rheumatic mitral stenosis. Chest 1977; 72: 752-56 4 Cheng TO: The click-murmur syndrome. A medical pendulum and a unifying concept. Chest 1978; 70:569-72 5 Cheng TO: Diastolic click in mitral valve prolapse. Arch Intern Med 1977; 137:696
A Clue to Pulmonary Embolism Obtained during Swan-Ganz Catheterization To the Editor:
An unusual clue to pulmonary embolism was noted in our laboratory during a recent right heart catheterization. CASE REPoRT
A 54-year-old woman was admitted with edema of the right arm and dyspnea. Evaluation revealed right subclavian vein thrombosis, malignant left pleural effusion, and a large pericardial effusion. Significant past history included repair of an atrial septal defect eight years previously. Pulmonary hypertension was present at that time. Right heart catheterization and pericardiocentesis was performed to determine the hemodynamic significance of the effusion and to obtain specimens for cytology. Under fluoroscopy, a Swan-Ganz catheter was advanced without difliculty via the right femoral vein to the right pulmonary artery. The pressure recordings were diagnostic of cardiac tamponade (elevated right heart diastolic pressures with equalization of right atrial, right ventricular diastolic, and intrapericardial preswoe; all 20 DUD Hg). The cardiac .output (thermodilu.ion) was dhninished (3.2 Llmin). We were not able to obtain pulmonary capillary wedge pressure. Sanguinous pericardJal fluid (1365 ml) was removed with dramatic improvement of the hemodynamic parameters. Within minutes the patient began to experience increasing respiratory difficulty with severe hypoxia unresponsive to mechanical ventilation. Attempts to repeat the measurement of cardiac output by thermodilution after pericardiocentesis were unsuccessful, despite performing the routine tests for catheter and computer malfunction and changing both the Swan-Ganz catheter and the cardiac output computer. Pulmonary angiography confirmed the presence of a massive embolus to the right main pulmonary artery. The patient was immediately placed on femoral-femoral cardiopulmonary bypass in the catheterization laboratory and taken immediately to surgery for emergency pulmonary embolectomy. Carcinomatosis involving the entire right hemothorax, including the pericardial surface, was documented.
=
The inability to measure cardiac output by thermodilution during Swan-Ganz catheterization may serve as a clue to pulmonary embolism if the usual causes of malfunction have been considered and excluded. Undoubtedly, the thermistor tip was lodged in the region of the clot, preventing accurate measurement of the temperature changes occurring during iced-water injection. Indeed, the SwanGanz tip was located in the same pulmonary artery as the
CHEST, 81: 4, APRIL, 1982
massive pulmonary embolus. One should consider pulmonary embolus in similar situations. ]tmnBt F. LewIs, M.D.; Timothy W. Anderson, M.D.; waUam H. Fennell, M.D.; and]tJmB8 B. Young, M.D., F.C.CP., Ben Toob General Hoapitlll, Houston
Reprint requests: D,. Young, 1200 Mouf'BUnd. 512D, Hous-
ton 77030
A Complication with Thermodilution Cardiac Outputs in Centrally-Placed Pulmonary Artery Catheters To the Editor:
The use of side-arm percutaneous central venous pressure introducer sets for the insertion of balloon-tipped pulmonary artery catheters is becoming increasingly popular. In addition to expediting the subclavian or intemal jugular insertion of the pulmonary artery catheter, they have the added feature of a side-arm fluid administration port. Recently, we performed a thermodilution cardiac.output study utilizing a 4-lumen Edwards thermodilution SwanGanz catheter inserted through a USCI "hemaquet" arterial/venous percutaneous catheter introducer sheath. Using the Edwards 9520A computer and 10 ml of iced injectate, cardiac output values of 24 L/min and 22 L/min were obtained. During the second injection, a retrograde surge of blood-tinged intravenous fluid was observed in the line infusing the side-port of the introducer sheath. Closing off the stopcock on this line before the next thermodilution injection resulted in cardiac outputs in the range of 9 L/min. These values were 'more consistent with the patient's clinical picture. The in vivo length of the pulmonary artery catheter measured at the hub of the introducer sheath was 42 CDl. Since the proximal line exit port is at 30 em, and the length of the introducer sheath and hub is 15 em, we concluded that the proximal port was still well within the lumen of the sheath. During injection of the 10 ml iced bolus, a significant portion of this volume was being forced retrograde into the side-arm intravenous line. As a result, a smaller volume of the injectate was actually being added to the venous blood, thus causing erroneously high cardiac output values. Closing off the stopcock to the side-arm intravenous line corrected this problem. Since pulmonary artery catheters placed either through the subclavian or internal jugular veins tend to have short in vivo catheter lengths, their placement within an introducer sheath increases the likelihood of the above problem occurring. Therefore, when performing thermodilution cardiac output studies through a pulmonary artery catheter utilizing an introducer sheath with a side-arm intravenous line, care must be taken to insure that the proximal port is beyond the tip of the sheath. Otherwise, fluid infusion through the side-arm should be from an infusion pump or the side-arm intravenous line should be temporarily turned off. Mark G. Bearss, B.S., R.R.T.; David N. ¥onutaB, M.S., R.R.T.; and William T. Allen, M.D., rac.r; Department of Cardiopulmonary Services, St. Joseph MerCfl H08fJital, Ann Arbor, Michigan
COMMUNICADONS TO THE EDITOR 527