The Father of Septal Myectomy for Obstructive HCM, Who Also Had HCM

Transcript

1 VOL. 67, NO. 24, 2016 JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY ISSN 0735-1097/$36.00 GY FOUNDATION 2016 BY THE AMERICAN COLLEGE OF CARDIOLO ª http://dx.doi.org/10.1016/j.jacc.2016.05.002 PUBLISHED BY ELSEVIER PAGE GUEST EDITORS ’ The Father of Septal Myectomy for Obstructive HCM, WhoAlsoHadHCM The Unbelievable Story b a William C. Roberts, MD Barry J. Maron, MD, internationally (5 – 7) .Now, > 50 years later, surgical ndrew Glenn Morrow, MD, was Chief of the myectomy continues to bene fi tthousandsofpatients Clinic of Surgery at the National Institutes A with HCM, resulting in reversal of heart failure of Health (NIH), Bethesda, Maryland, from symptoms and restoration of normal daily activity in ). During that Figure 1 1953 until his death in 1982 ( 12) – (5,8 almost 90% ,aswellasenhancedsurvival time, he established an international training pro- .Inguide- consistent with the general population (5) gram for future cardiac surgeons, and among many (10) , (11) and consensus recommendations lines entricular septal myec- other accomplishments, the v myectomy remains the primary treatment option for tomy operation for obstructive hypertrophic cardio- most patients with severe drug-refractory heart myopathy (HCM) (1,2) . failure symptoms, becoming the safest open heart fi rst surgeon to perform the myectomy He was the procedure when performed in experienced centers operation to relieve left ventricular (LV) out fl ow . (6) (mortality, 0.3%) obstruction in patients experiencing inadequate symptom response to medical treatment, a proce- dure which he designed, which he performed on 299 .Asmall (1,2) patients, and which bears his name amount of muscle is resected from the basal septum FIGURE 1 Andrew Glenn Morrow, 1963 to widen the LV out (3) andabolishsystolic ow tract fl anterior motion of the mitral valve, the mechanism responsible for subaortic obstruction. Dr. Morrow also was fi rsttoreportobstructionasdueto cient to impede LV muscular hypertrophy suf fi . out fl ow (4) The Morrow operation (now modi fi ed by extended muscular resection) was introduced in the early 1960s at the NIH and quickly spread to major institutions in the United States, including Mayo Clinic, and a From the Hypertrophic Cardiomyopathy Institute, Division of Cardiol- ogy, Tufts Medical Center, Boston, Massachusetts (formerly at Minne- apolis Heart Institute Foundation, Hypertrophic Cardiomyopathy Center, b Minneapolis, Minnesota); and the Baylor Heart and Vascular Institute, Dallas, Texas. Both authors have reported that they have no relationships relevant to the contents of this paper to disclose.

2 JACC VOL. 67, NO. 24, 2016 2901 Maron and Roberts 3 – JUNE 21, 2016:2900 Page ’ Guest Editors HCM AND GLENN MORROW S ILLNESS ’ Morrow Family Pedigree FIGURE 2 It is not widely known that Dr. Morrow himself had ? ? I. HCM, with this diagnosis substantiated by an 1 34 2 fi aggressive form of the disease identi ed in 2 of his (83) (65) (60) (91) (83) ). Dr. Eugene Braunwald, Chief of Figure 2 children ( II. Cardiology, was a close friend and colleague of 3 1 2 rst fi Dr. Morrow. While collaborating at the NIH on the (66) (52) (63) major clinical description of HCM (13) , Dr. Morrow III. Gene, would you examine my “ asked Dr. Braunwald: 12 34 (14) ” heart? .Solelybyprecordialauscultation,and (16) (23) (21) (20) fi nding of a systolic ejection recognizing the classic murmur due to LV out ow obstruction, Dr. Braunwald fl Squares indicate men; indicate women; arrow indicates circles made the diagnosis of obstructive HCM at age 40 years proband (Dr. Morrow, I.1.); solid symbols indicate hypertrophic indicates deceased. slash cardiomyopathy; and IHSS ” at that time) (13,14) . This diagnosis “ (called was impressive for several reasons: 1) it occurred in 1961, in the pre-echocardiographic era; and 2) it was preserved LV hypertrophy and systolic function made with auscultation when diagnostic criteria ,nondilatedLV,andbia- (15) (ejection fraction, 67%) for this complex disease were just being formulated. trial enlargement, characteristic of a novel subgroup fi ed Dr. Morrow was probably the 25th clinically identi of transplant candidates . She underwent suc- (16) (13,14) HCM patient . cessful heart transplant at age 51 years, and has been Dr. Morrow experienced exertional dyspnea and genotyped to a pathogenic beta-myosin heavy chain near-syncope almost daily in the morning, multiple > G; variant mutation (MYH7) (DNA coding c.485A episodes of syncope, atrial fi brillation, and ulti- pTyr162Cys [y162c]). mately, embolic stroke. He refused beta-blocker therapy, cardiac catheterization, and consideration schildrenisa63-year- ’ Another of Dr. Morrow SON. for septal myectomy. Dr. Morrow died suddenly at old man who has obstructive HCM (septal thickness, only 60 years of age in his home. His heart was Figure 2 21 mm) ( ). For many years, he has been berts with the permission recovered by Dr. William Ro encumbered by recurrent episodes of syncope, s wife. At autopsy, cardiac morphologic of Dr. Morrow ’ brillation (left atrium, 48 mm) symptomatic atrial fi fi ndings were typical of HCM with asymmetric septal hypertrophy, myocyte disarray, myocardial FIGURE 3 The Heart of Dr. Morrow crovascular abnormalities scarring, and structural mi ). ( Figure 3 THE MORROW FAMILY DISEASE The Morrows were married for 37 years and had 3 children, including 2 with an unusually aggressive form of HCM. One child is a 52-year-old woman who DAUGHTER. was diagnosed with HCM at age 21 with a heart murmur due to mitral valve systolic anterior motion ). and ventricular septal hypertrophy ( Figures 2 and 4 For many years, she experienced symptoms of heart failure and limited physical activity, refractory to pharmacological agents, ch ronic dual-chamber pacing (A) The ascending aorta (Ao) is free of atherosclerotic plaque, with a Weight, 645 g. structurally normal aortic valve, nondilated left ventricular (LV) cavity, and greatly dilated , and also radiofrequency ablations that were un- (15) left atrium (LA). Ventricular septal (VS) thickness is 20 mm, asymmetrically hypertrophied successful in reducing symptomatic episodes of atrial fi c deposit with respect to LV free wall (FW). Behind posterior mitral lea fl et is a large calci brillation. fi (arrow) , frequent in older patients. Several small scars are evident in the VS and at the Although initially with a subaortic gradient of 70 (arrowheads) In the subaortic region of the hypertrophied septum is a fi brous (B) . apex mm Hg, she evolved to a nonobstructive state with fl in apposition to the thickened anterior mitral lea (arrowheads) plaque et (AML), evidence ow obstruction. RV ¼ right ventricle. of prior out fl end-stage heart failure (New York Heart Association functional class III to IV), associated with both

3 JACC VOL. 67, NO. 24, 2016 2902 Maron and Roberts – JUNE 21, 2016:2900 3 Page ’ Guest Editors The Explanted Heart of Daughter FIGURE 4 The VS thickness is 23 mm, asymmetrically hypertrophied with respect to LV FW, and with a nondilated LV cavity. (A) Weight 510 g. adjacent to the thickened AML, and prior evidence of mitral valve systolic (white arrowheads) brous plaque fi Septal endocardial (B) ; (blue) brosis Typical disorganized myocyte arrangement in septum, and areas of interstitial (C) fi anterior motion and obstruction. (D) 400).  myocardial scarring was absent ( brous thickening ( fi Intramural coronary artery with disrupted media and intimal 100).  Figure 3 . Masson trichrome stain. Abbreviations as in is reported here to complete the historical record. As requiring cardioversion, and 1 transient ischemic ected Dr. Braunwald re fl (14) ,shouldthisscenario attack. At age 57 years, he developed severe pro- have been encountered on television or in the gressive heart failure due to LV out ow obstruction fl movies, it would be considered impossible or ridicu- (gradient, 55 mm Hg). He underwent successful sur- lous, that is, one colleague diagnosing the other while gical septal myectomy with symptomatic relief, they are both collaborating on the description of the remarkably with the sam eoperationhisfather same new disease, which was then considered had developed and promoted 5 decades earlier (1,2) . extraordinarily rare. His 21-year-old daughter has HCM, completing a In addition, several important facets of HCM are 3-generation disease transmission. underscored here, that is, an autosomal dominant disease with great heterogeneity in expression, the PERSPECTIVES brillation, and contemporary fi importance of atrial The saga of Dr. Glenn Morrow, HCM, and the Morrow . The particularly (17) management considerations family is of unique interest to cardiovascular medi- aggressive clinical variants reported here in Dr. cine, and also one which has been largely unknown. It Morrow and his offspring are considered somewhat

4 JACC VOL. 67, NO. 24, 2016 2903 Maron and Roberts 3 – JUNE 21, 2016:2900 Page ’ Guest Editors symptoms in the son and reversed by the very oper- unusual within the broad HCM disease spectrum, ation designed by and named for his father. most often associated with normal life expectancy (12,17) . Indeed, all 3 in- without major disability REPRINT REQUESTS AND CORRESPONDENCE: Dr. dividuals were affected by a progressive disease Barry J. Maron, HCM Institute, Division of Cardiology, fi brillation (with course involving repetitive atrial Tufts Medical Center, 800 Washington Street #70, thromboembolism in the father), recurrent syncope Boston, Massachusetts 02111. E-mail: [email protected] (both males), heart trans plant (the daughter), and gmail.com . subaortic obstruction causing severe heart failure REFERENCES 1. Morrow AG, Lambrew CT, Braunwald E. cardiomyopathy centers in the management of with translation into contemporary cardiovascular Idiopathic hypertrophic subaortic stenosis. II. dynamic subaortic obstruction. J Am Coll Cardiol . – medicine. J Am Coll Cardiol 2014;64:83 99 Operative treatment and the results of pre- and – 2015;66:1307 8 . 13. Braunwald E, Lambrew CT, Rockoff SD, et al. postoperative hemodynamic evaluations. Circula- 7. Dearani JA. Septal myectomy remains the gold Idiopathic hypertrophic subaortic stenosis. 1. A 51 tion 1964;30 Suppl 4:120 . – standard. Eur Heart J 2012;33:1999 – 2000 . description of the disease based upon an analysis 2. Morrow AG, Reitz BA, Epstein SE, et al. Opera- of 64 patients. Circulation 1964;30 Suppl 4: Woo A, Williams WG, Choi R, et al. Clinical and 8. tive treatment in hypertrophic subaortic stenosis. – 119 . 3 echocardiographic determinants of long-term Techniques, and the results of pre and post- survival after surgical myectomy in obstructive Maron BJ, Braunwald E. Eugene Braunwald, 14. operative assessments in 83 patients. Circulation hypertrophic cardiomyopathy. Circulation 2005; MD and the early years of hypertrophic cardio- 1975;52:88 – . 102 41 – 111:2033 . myopathy: a conversation with Dr. Barry J. Maron. 3. Spirito P, Maron BJ, Rosing DR. Morphologic Am J Cardiol 2012;109:1539 – 47 . Smedira NG, Lytle BW, Lever HM, et al. Current 9. determinants of hemodynamic state after ven- effectiveness and risks of isolated septal myec- Maron BJ, Josephson ME. Long-term conse- 15. tricular septal myotomy-myectomy in patients tomy for hypertrophic obstructive cardiomyopa- quences of the right ventricular pacing mania of with obstructive hypertrophic cardiomyopathy: M – . thy. Ann Thorac Surg 2008;85:127 33 the 1990s for obstructive hypertrophic cardiomy- mode and two-dimensional echocardiographic opathy. Am J Cardiol 2014;113:191 . 2 – Gersh BJ, Maron BJ, Bonow RO, et al. 2011 10. . – assessment. Circulation 1984;70:984 5 ACC/AHA guideline for the diagnosis and treat- 16. Rowin EJ, Maron BJ, Kiernan MS, et al. Morrow AG, Braunwald E. Functional aortic 4. ment of hypertrophic cardiomyopathy: American Advanced heart failure with preserved systolic stenosis; a malformation characterized by resis- College of Cardiology Foundation/American Heart function in nonobstructive hypertrophic cardio- fl tance to left ventricular out ow without anatomic Association task force on practice guidelines. J Am myopathy: under-recognized subset of candidates 9 . obstruction. Circulation 1959;20:181 – 38 Coll Cardiol 2016;58:2703 . – for heart transplant. Circ Heart Fail 2014;7: 967 . 75 – 5. Ommen SR, Maron BJ, Olivotto I, et al. Long-term 11. Maron BJ, McKenna WJ, Danielson GK, et al. effects of surgical septal myectomy on survival in American College of Cardiology/European Society Maron BJ, Rowin EJ, Casey SA, Maron MS. 17. patients with obstructive hypertrophic cardiomy- of Cardiology clinical expert consensus document How hypertrophic cardiomyopathy became a opathy. J Am Coll Cardiol 2005;46:470 . 6 – on hypertrophic cardiomyopathy. J Am Coll Car- contemporary treatable genetic disease with low – . diol 2003;42:1687 713 mortality: shaped by 50 years of clinical research Maron BJ, Dearani JA, Ommen SR, et al. 6. and practice. JAMA Cardiol 2016 Mar 2 [E-pub Low operative mortality achieved with surgical 12. Maron BJ, Ommen SR, Semsarian C, et al. . ahead of print] septal myectomy: role of dedicated hypertrophic Hypertrophic cardiomyopathy: present and future,

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