Ann Thorac Surg 1996;61:1829-32
patients, only 17% of the patients with C C A M were more than 6 m o n t h s of age [6]. The presentation in older patients is usually that of recurrent p u l m o n a r y infections. In addition to the typical respiratory lining cells and mucin-secreting cells, the cysts in older patients d e m o n strate an inflammatory c o m p o n e n t not found in infants. The characteristic cystic changes found in CCAM usually involve a single lobe. Of the 153 cases collected by Cloutier and associates [6], there were only 27 cases of multilobar involvement including a single report of bilateral disease. All three lobes in our patient h a d cystic changes, with the most severe cystic disease located in the m i d d l e and lower lobes. Due to the associated p a t h o logic findings, every a t t e m p t was m a d e to preserve any normal right lung. Browdie and colleagues [71 have successfully p e r f o r m e d n o n a n a t o m i c resections of C C A M in infants, with long-term follow-up showing increased function in the r e m a i n i n g lung. Certainly in infants, the potential for lung growth exists, but w h e t h e r significant growth could occur in the adult to offset the i m m e d i a t e space p r o b l e m s created is unclear. The constellation of cavernous transformation of the portal and splenic veins, vertebral anomalies, sacral mass, and cystic a d e n o m a t o i d malformation of the lung is unusual. Other anomalies associated with CCAM have b e e n described in up to 18% of patients [6]. These have most c o m m o n l y been renal agenesis a n d cardiac anomalies. Congenital cystic a d e n o m a t o i d malformation is an u n c o m m o n cause of p u l m o n a r y pathology in the adult. Resection usually results in i m p r o v e d respiratory, function. In this case, the split function perfusion study was very helpful in d e t e r m i n i n g operability. A s s o c i a t e d anomalies are c o m m o n a n d m a y influence perioperative management.
References 1. Han YM, Lee DK, Lee SY, et al. Adult presentation of congenital cystic adenomatoid malformation of the lung: a case report. J Korean Med Sci 1994;9:86-91. 2. Akiba T, Yamazaki Y, Yasukawa S, Yuno S, Yoshida T, Sakurai K. A case of congenital cystic adenomatoid malformation of the lung in an 18 year old male. Nippon Kyobu Geka Gakkai Zasshi 1992;40:161-4. 3. Pulpeiro JR, Lopez I, Sotelo T, Ruiz JC, Garcia-Hidalgo E. Congenital cystic adenomatoid malformation of the lung in a young adult. Br J Radiol 1987;60:1128-30. 4. Sagawa H, Ebihara Y, Kuwabara T, Sasaki T, Yanagisawa M, Kidokoro T. Two adult cases of pulmonary congenital cystic adenomatoid malformation. Nippon Kyobo Shikkan Gakkai Zasshi 1985;23:593-8. 5. Hlnick DH, Naidich DP, McCauley DI, et al. Late presentation of congenital cystic adenomatoid malformation of the lung. Radiology 1984;151:569-73. 6. Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest 1993;103:761-4. 7. Browdie D, Todd D, Agnew R, Rosen W, Beardmore H. The use of nonanatomic resection in infants with extensive congenital adenomatoid malformation of the lung. J Thorac Cardiovasc Surg 1993;105:732-6. © 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
CASE REPORT KAMEDAET AL MITRAL ANNULOPLASTYFOR DCM
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Annuloplasty for Severe Mitral Regurgitation Due to Dilated Cardiomyopathy Yoichi Kameda, MD, Soichiro Kitamura, MD, Kanji Kawachi, MD, a n d Tetsuji Kawata, MD Department of Surgery III, Nara Medical College, Nara, Japan
We present a 74-year-old woman who underwent corrective annuloplasty for severe mitral regurgitation due to dilated cardiomyopathy. Postoperatively, congestive heart failure improved, with her New York Heart Association status changing from IV to II, although her cardiac functional improvement was minimal. Severe mitral regurgitation may be the indication for annuloplasty in symptomatic patients with dilated cardiomyopathy when cardiac transplantation is not indicated.
(Ann Thorac Surg 1996;61:1829-32) ilated c a r d i o m y o p a t h y (DCM) is frequently accomp a n i e d by mitral regurgitation (MR) and complicates the medical t r e a t m e n t for congestive heart failure (CHF). Cardiac transplantation is a definitive t r e a t m e n t for this end-stage feature of DCM; however, m a n y patients are excluded from its indication because of various reasons including a d v a n c e d age. W e p r e s e n t an elderly patient with severe MR a n d CHF who s u b s e q u e n t l y u n d e r w e n t mitral annuloplasty. Postoperatively, she exp e r i e n c e d some i m p r o v e m e n t in her s y m p t o m s and h e m o d y n a m i c s after reduction of MR from grade 4/4 to 1/4. W e p r e s e n t in this report the alterations of left ventricular function.
D
A 74-year-old w o m a n was a d m i t t e d for severe CHF (New York Heart Association [NYHA] class IV) despite maximized medical therapy. She h a d b e e n d i a g n o s e d as having heart disease at 54 years of age, a n d h a d suffered from palpitations and d y s p n e a on exertion since she was a p p r o x i m a t e l y 66 years of age. She manifested o r t h o p n e a at the end of F e b r u a r y 1992 a n d was a d m i t t e d to the hospital on March 4. Her o r t h o p n e a was i m p r o v e d soon after admission by intravenous administration of diuretics a n d catecholamine, but the NYHA class IV heart failure continued despite m a x i m i z e d medical therapy. Echocardiography revealed severe diffuse hypokinesis a n d dilatation of the left ventricle (Table 1). D o p p l e r e c h o c a r d i o g r a p h y d e m onstrated a grade 4/4 MR. Cardiac catheterization also revealed a dilated and hypokinetic left ventricle with completely normal coronary arteries (Table 1; Fig 1). The calculated regurgitant fraction was 0.62, and a grade 4/4 Accepted for publication Dec 4, 1995. Address reprint requests to Dr Kitamura, Department of Surgery III, Nara Medical College, 840 Shijo-cho,Kashihara, Nara, 634, Japan. 0003-4975/96[$15.00 PII S0003-4975(96)00004-8
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CASE REPORT K A M E D A ET A L MITRAL ANNULOPLASTY FOR DCM
Ann Thorac Surg 1996;61:1829-32
Table 1. Cardiac Catheterization and Echocardiographic Findings Postoperative Variable
Preoperative
30 Days
2 8 Months Months
Catheterization Mean RAP 1 1 (mm Hg) RVP (mm Hg) a 28•2 18/2 PAP (mm Hg) ~ 28•8 (16) 18/4 (10) Mean PAWP 11 4 (mm Hg) LVP (mm Hg)" 110/10 90•4 AoP (ram Hg) a 110160 (78) 90156 (70) Heart rate (beats/ 97 82 rnin) Cardiac index 2.48 2.41 (L " min -1 " m 2) Stroke index 29 30 (mL/m 2) LVEDVI (inL/m 2) 276 229 LVESVI (mL/m 2) 203 202 LVEF 0.26 0.12 Regurgitant fraction 0.62 0.11 Echocardiography LAD (mm) 53 ,, 44 LVD diastolic/ 74/65 .. 66/63 systolic (mm) Fractional shortening 0.12 .. 0.05
Her postoperative course was uneventful with the aid of catecholamines for 7 days, a n d her functional class i m p r o v e d to NYHA class II. Postoperative cardiac catheterization, p e r f o r m e d 30 days after the procedure, revealed significant i m p r o v e m e n t in mitral regurgitation to grade 1/4 (see Fig 1), a n d the calculated regurgitant fraction was 0.11 (see Table 1). The left ventricular enddiastolic volume was r e d u c e d by 20%, but the endsystolic volume r e m a i n e d the same a n d the ejection fraction decreased from 0.26 to 0.12 regardless of the clinical i m p r o v e m e n t . She was discharged on the 35th postoperative day. S u b s e q u e n t e c h o c a r d i o g r a p h y d e m onstrated i m p r o v e m e n t in the left ventricular fractional shortening from 0.06 in the second postoperative m o n t h to 0.09 in the eighth postoperative month, and the MR r e m a i n e d grade 1/4. A l t h o u g h 1 year after the operation her s y m p t o m s were still consistent with NYHA class II, her condition gradually deteriorated with renal failure a n d she died 21 m o n t h s postoperatively. Comment
44 68162 0.09
a D a t a a r e e x p r e s s e d as s y s t o l i c / e n d - d i a s t o l i c or systolic/diastolic ( m e a n ) . A o P - aortic p r e s s u r e ; L A D ~ left atrial d i m e n s i o n ; L V D = left ventricular dimension; LVED(S)VI left v e n t r i c u l a r e n d - d i a s t o l i c (end-systolic) v o l u m e index; LVEF - left v e n t r i c u l a r ejection fraction; LVP - left v e n t r i c u l a r p r e s s u r e ; P A P = p u l m o n a r y arterial p r e s s u r e ; P A W P = p u l m o n a r y arterial w e d g e p r e s s u r e ; RAP r i g h t atrial pressure; RVP right ventricular pressure.
mitral regurgitation was observed. Right ventricular end o m y o c a r d i a l biopsy was performed, and the findings were consistent with a diagnosis of DCM (Fig 2). W e considered that her heart failure was w o r s e n e d b y MR a n d we could not anticipate i m p r o v e m e n t in her CHF from further medical treatments. Therefore, we estim a t e d a s u b s e q u e n t life span of months. The patient a n d her family were i n f o r m e d of her prognosis, the operation risks, a n d the potential benefits of mitral annuloplasty. The operation was p e r f o r m e d in July 1992 via a m e d i a n sternotomy using m o d e r a t e l y h y p o t h e r m i c c a r d i o p u l m o nary b y p a s s with i n d u c e d ventricular fibrillation. Left atriotomy revealed neither torn mitral valve chordae nor m y x o m a t o u s degeneration. The mitral annular d i a m e t e r was dilated to 34 ram, and there were no abnormalities of the subvalvular apparatus. Therefore, the cause of MR was a s s u m e d to be mitral annular e n l a r g e m e n t associated with papillary muscle dysfunction. Mitral annuloplasty, using a 28-mm Carpentier annuloplasty ring, was performed. Significant reduction of MR was confirmed by t r a n s e s o p h a g e a l D o p p l e r echocardiography. A pacem a k e r lead was prophylactically i m p l a n t e d on the left ventricular surface because of the presence of paroxysmal complete atrioventricular block before the operation.
Although extensive medical therapy, such as vasodilator t h e r a p y and /3-blocker administration, has p r o l o n g e d survival, the prognosis of patients with CHF due to DCM r e m a i n s poor. The association of significant MR in this condition is a poor prognostic sign [1, 21. At present, heart transplantation is the therapeutic a p p r o a c h associated with the best survival rate in patients with severely i m p a i r e d cardiac function; nevertheless, its indications are limited. Our patient's age of 74 years p r e c l u d e d her from being a candidate for transplantation. Therefore, we p e r f o r m e d mitral annuloplasty in an a t t e m p t to improve her CHF with the abolition of MR. Although there are m a n y reports about c o m b i n e d mitral valve operation a n d coronary b y p a s s grafting for ischemic cardiomyopathy, reports of surgical t r e a t m e n t for mitral regurgitation due to DCM without coronary artery disease are rare. Two cases of mitral valve replacem e n t have b e e n r e p o r t e d by Bolen a n d A l d e r m a n [3]. In I case, the patient died i m m e d i a t e l y after the operation. In the other case, the patient died 9 months p o s t o p e r a tively after having d e m o n s t r a t e d minimal clinical b e n e fits. Kenny a n d colleagues [4] have r e p o r t e d 2 cases of mitral ring valvuloplasty for DCM, although details regarding the clinical courses were not provided. Recently Bolling and associates [5] r e p o r t e d 16 cases of mitral valve reconstruction for end-stage cardiomyopathy. They e m p h a s i z e d that there were three late deaths and the r e m a i n i n g 13 patients had i m p r o v e m e n t of s y m p t o m s from NYHA class IV p r e o p e r a t i v e l y to I or II p o s t o p e r a tively. The m e a n ejection fraction increased from 0.18 -+ 0.05 to 0.24 _+ 0.10, with statistical significance. This i m p r o v e m e n t of ejection fraction is surprisingly good. In analyzing their data [5], the reduction of end-systolic volume is greater than that of end-diastolic volume resulting in an increase in stroke volume, which suggests i m p r o v e m e n t of contractility by mitral valve repair in their series. This m a y have occurred b e c a u s e of the i m p r o v e m e n t of heart failure s e c o n d a r y to MR, the use of
Ann Thorac Surg 1996;61:1829-32
CASE REPORT KAMEDA ET AL MITRAL ANNULOPLASTY FOR DCM
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Fig 1. Preoperative (left) and postoperative (right) left ventriculography. The upper is an end-diastolic and the lower is an end-systolic view. Preoperatively, a grade 4/4 mitral regurgitation was present. Postoperatively, the mitral regurgitation improved to grade 1/4.
flexible annuloplasty rings, or performance of additional coronary bypass grafting in some patients. In our patient, the MR improved from grade 4/4 to 1/4 and the regurgitant fraction decreased from 0.62 to 0.11
Fig 2. Right ventricular endomyocardial biopsy specimen with findings compatible with dilated cardiomyopathy. Hypertrophic and atrophic myocardial cells, interstitial fibrosis, and infiltration by fatty cells are visible.
postoperatively. The left ventricular end-diastolic volume also decreased by 47 mL/m 2, although there was little change in the end-systolic volume and forward stroke volume. Consequently, in our patient the left ventricular ejection fraction decreased from 0.26 to 0.11, and the improvement in cardiac function was minimal by annuloplasty alone, which usually increases afterload of a poorly contracting left ventricle (afterload mismatch). In the late postoperative period, however, echocardiography revealed some improvement of the left ventricular fractional shortening from 0.06 at 2 months postoperatively to 0.09 at 8 months postoperatively. Also, clinically the operation was effective in improving her CHF and symptomatology. Although we thought that her survival would be limited to less than 6 months before the operation, she continued to live for approximately 1.5 years with a cardiac condition consistent with NYHA class II. Considering this case together with those reported by Bolling and associates [5], mitral annuloplasty may offer some improvements in clinical symptoms and survival period in patients with significant MR due to DCM not indicated for heart transplantation.
References 1. Blondheirn DS, Jacobs LE, Kotler MN, Costacurta GA, Parry WR. Dilated cardiomyopathy with mitral regurgitation: de-
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4.
5.
CASEREPORT MYERSAND COOPWOOD MITRALVALVEREPLACEMENT
creased survival despite a low frequency of left ventricular thrombus. Am Heart J 1991;122:763-71. Romeo F, Pelliccia F, Cianfrocca C, et al. Determinants of end-stage idiopathic dilated cardiomyopathy: a multivariate analysis of 104 patients. Clin Cardiol 1989;12:387-92. Bolen JL, Alderman EL, Ventriculography and hemodynamic features of mitral regurgitation of cardiomyopathic, rheumatic and nonrheumatic etiology. Am J Cardiol 1977;39: 177-83. Kenny J, Cohn L, Shemin R, Collins JJ, Plappert M, Sutton MG. Doppler echocardiographic evaluation of ring mitral valvuloplasty for pure mitral regurgitation. Am J Cardiol 1987;59:341-45. Bolling SF, Deeb M, Brunsting LA, Bach DS. Early outcome of mitral valve reconstruction in patients with end-stage cardiomyopathy. J Thorac Cardiovasc Surg 1995;109:676-83.
Mitral Valve Replacement in the Transplanted Heart John C. Myers, MD, and Joseph B. Coopwood, MD Department of Cardiothoracic Surgery, Wilford Hall USAF Medical Center, Lackland Air Force Base, Texas
Because of the scarcity of donor hearts, conventional operations on heart allografts are now being performed in lieu of retransplantation. Our experience with mitral valve replacement in the orthotopically transplanted heart is presented, supporting the utility of conventional operations when indicated.
(Ann Thorac Surg 1996;61:1832-3) s donor organs become increasingly scarce, cardioA thoracic transplant surgeons need to consider conventional operations to correct abnormalities in previously transplanted hearts. Accelerated atherosclerosis in heart allografts and valvular dysfunction caused by repeated endomyocardial biopsy are well described and may lead to life-threatening conditions that require prompt surgical therapy. Finding a suitable donor heart for retransplantation in such a situation may not be a tenable option for these patients. Conventional operations on heart allografts therefore are now being attempted in lieu of retransplantation. Indeed, mitral valve replacement [1], tricuspid valve replacement/repair [2, 31, aortic valve replacement [4, 5], and coronary artery bypass grafting [6] have all been performed successfully in transplanted hearts. Here, we present our experience with mitral valve replacement in the orthotopically transplanted heart. The patient is a 47-year-old w o m a n with a history of dysfibdnogenemia and hypercoagulable state in w h o m severe ischemic cardiomyopathy (ejection fraction -0.18} Accepted for publication Dec 18, 1995. Address reprint requests to Dr Myers, WHMC/PSST, 59 MDW AETC, 2200 Bergquist Dr, Ste 1, Lackland AFB,TX 78236-5300.
Published by Elsevier Science Inc
Ann Thorac Surg 1996;61:1832-3
developed after a myocardial infarction in 1980. In June 1992 she underwent orthotopic heart transplantation, which was complicated by a single episode of acute rejection and sudden cardiac death in the early posttransplantation period. Her immunosupressive regimen has consisted of FK506, azathioprine, and prednisone after cyclosporine administration was discontinued secondary to paresthesias. Also of note, this patient had undergone several endomyocardial biopsies via the left side of the heart instead of the right secondary to thrombosed femoral and neck veins due to her prior hypercoagulable state. The patient presented to our facility in August 1994 with complaints of decreasing exercise tolerance and increasing shortness of breath. Cardiac catheterization performed earlier in June 1994 had shown irregular two-vessel coronary artery disease in the left anterior descending and left circumflex arteries, normal left ventricular function (ejection fraction, 0.70), and severe mitral regurgitation. It was thought that the mitral insufficiency was a result of iatrogenic trauma to the valve during the patient's repeated left-sided endomyocardial biopsies. Shortly after admission, transesophageal echocardiography was performed, which showed severe mitral regurgitation with severe prolapse of both the anterior and posterior leaflets and a torn posterior mitral valve chorda tendinae. Because of the patient's severe valvular dysfunction and progressive symptoms, urgent mitral valve replacement was deemed appropriate. The patient gave informed consent and was brought to the operating room 26 months after transplantation. A redo sternotorny was performed, and the patient was placed on cardiopulmonary bypass without complication. Myocardial protection was produced by anterograde cold blood cardioplegia. After the heart was arrested, the mitral valve was exposed using a transseptal approach via the right atrium. Operative findings included multiple torn chordae tendineae involving both the anterior and posterior leaflets. The anterior leaflet was then completely excised, the flail chordae were trimmed, and a 25-ram Medtronic-Hall (Medtronic, Inc, Minneapolis, MN) mechanical valve was seated without difficulty using supraannular pledgeted horizontal mattress sutures. Valve function during intraoperative testing was excellent. Closure of the atrial septum, deairing of the left ventricle, and closure of the right atrium all took place without problem. Weaning from cardiopulmonary bypass was then accomplished with the aid of a dobutamine drip. The remainder of the procedure was uneventful. The patient did well" postoperatively except for an episode of acute renal failure that resolved with an increase in diuretic therapy and a decrease in FK506 dosing. Renal function was back to baseline by postoperative day 4 and the patient was subsequently discharged to home on postoperative day 12 and is doing well. 0003-4975/96l$15.00 PII S0003-4975(96)00061-6