The optimal treatment strategy for patients with symptomatic hypertrophic obstructive cardiomyopathy
(HOCM) and end-stage liver disease (ESLD) is not well defined. Although medical
management is the accepted first line treatment, patients who are unresponsive to medication
require further interventions. Since ESLD patients have a high operative risk for surgical
myomectomy, alcohol septal ablation (ASA) emerges as a good alternative in these cases. The
timing of ASA in relation to liver transplantation is still unclear. We report here on the first
case of an orthotopic liver transplant-recipient undergoing ASA and the second of a cirrhotic
patient requiring ASA as a bridge to liver transplantation. Both patients had a good clinical
outcome and we argue that ASA in HOCM patients should be driven by symptom onset, and
that in the asymptomatic patient it can be safely deferred until after liver transplantation.
(Cardiol J 2008; 15: 74-79

Aqel, Raed A.

Bravo, Paco E.

Bynon, John S.

Hage, Fadi G.

Zoghbi, Gilbert J.

English

Via Medica Journals

CASE REPORTCardiology Journal2008, Vol. 15, No. 1, pp. 74–79Copyright © 2008 Via MedicaISSN 1897–559374 www.cardiologyjournal.orgAddress for correspondence: Raed A. Aqel, MD, FACCBDB 383, 1530 3rd AVE SBirmingham, AL 35294-0007Tel: 205 934 7898, fax: 205 558 4714e-mail: raed.aqel@med.va.govReceived: 21.08.2007 Accepted: 21.11.2007Hypertrophic obstructive cardiomyopathyin liver transplant patientsFadi G. Hage, Paco E. Bravo, Gilbert J. Zoghbi, John S. Bynon and Raed A. AqelUniversity of Alabama at Birmingham, Birmingham, AL, USAAbstractThe optimal treatment strategy for patients with symptomatic hypertrophic obstructive cardio-myopathy (HOCM) and end-stage liver disease (ESLD) is not well defined. Although medicalmanagement is the accepted first line treatment, patients who are unresponsive to medicationrequire further interventions. Since ESLD patients have a high operative risk for surgicalmyomectomy, alcohol septal ablation (ASA) emerges as a good alternative in these cases. Thetiming of ASA in relation to liver transplantation is still unclear. We report here on the firstcase of an orthotopic liver transplant-recipient undergoing ASA and the second of a cirrhoticpatient requiring ASA as a bridge to liver transplantation. Both patients had a good clinicaloutcome and we argue that ASA in HOCM patients should be driven by symptom onset, andthat in the asymptomatic patient it can be safely deferred until after liver transplantation.(Cardiol J 2008; 15: 74–79)Key words: cardiomyopathy, liver transplantation, liver failure, alcohol,hypertrophy, ablationIntroductionHypertrophic obstructive cardiomyopathy(HOCM) is a heterogeneous disorder, affectingpatients of all ages. It has a diverse clinical coursewhere some individuals remain asymptomaticthroughout life while others develop severe symp-toms of heart failure, angina or syncope, and a mi-nority present with sudden cardiac death. The leftventricular outflow gradient is an independent pre-dictor of HOCM-related death, progression to NewYork Heart Association (NYHA) class III or IV heartfailure, and for death from heart failure or strokewhen it exceeds 30 mm Hg [1]. The optimal treat-ment strategy for patients with symptomatic HOCMand end-stage liver disease (ESLD) is not well defined.ESLD patients have a high operative risk for surgicalmyomectomy [2–5]. Alcohol septal ablation (ASA)emerges as a good alternative to surgical myomecto-my in these patients. The presence of symptomaticHOCM is considered a contraindication for liver trans-plantation in ESLD [6]. We report on two patients withESLD who underwent ASA, the first secondary toworsening symptoms after liver transplant and thesecond as a bridge to liver transplantation.Case No. 1A 58-year-old white female with HOCM andESLD secondary to longstanding autoimmune hep-atitis presented for evaluation for liver transplan-tation due to deteriorating hepatic function. She wasrelatively asymptomatic from the cardiac standpointwith no angina, heart failure or syncopal symptoms.On physical examination, she was jaundiced but hadno jugular venous distention (JVD). The carotidupstrokes were bifid, the first and second heartsounds were normal, and a fourth heart sound was75Fadi G. Hage et al., Hypertrophic obstructive cardiomyopathy and liver transplantswww.cardiologyjournal.orgpresent. A 3/6 mid- to late-peaking outflow murmurwas heard across the precordium. Lungs were clear,and there was no peripheral oedema. Electrocardio-graphy showed a left bundle branch block and leftventricular hypertrophy. A transthoracic echocardi-ogram (TTE) showed a left ventricular outflow tract(LVOT) gradient of 91 mm Hg, interventricular sep-tum (IVS) of 15 mm, left ventricular posterior wall(LVPW) of 14 mm and mild systolic anterior mo-tion (SAM). Cardiac catheterization showed a LVOTgradient of 60 mm Hg at rest, a hyperdynamic leftventricle with a left ventricular ejection fraction(LVEF) of 80%, and angiographically normal-ap-pearing coronaries. Since her cardiac symptomatol-ogy was stable on beta-blockers, she was consid-ered to be a suitable candidate for orthotopic livertransplantation, which she underwent with no ad-verse cardiovascular events. Within one year of herliver transplantation, she developed lower extrem-ity oedema and angina despite adequate medicaltherapy. TTE then showed an LVOT gradient of 99mm Hg, IVS of 20 mm and LVPW of 16 mm. Surgi-cal myomectomy versus ASA was discussed withthe patient, who opted for ASA. This was performedwith an infusion of 3 cc of 100% ethanol into the firstand second septal arteries under myocardial con-trast echocardiography and fluoroscopic guidance.The left ventricle/aorta (LV/Ao) gradient was 100mm Hg at rest and 200 mm Hg after a prematureventricular contraction (PVC), but decreased to 10mm Hg and 30 mm Hg at rest and after a PVC, re-spectively, post-ablation (Fig. 1). She developedcomplete heart block on the second day after theprocedure and required a permanent pacemaker.She became symptom free post ASA and remainedwithout angina or heart failure symptoms one yearlater. TTE then showed reduction of the IVS andLVPW diameters to 16 and 15 mm, respectively,with a preserved LVEF and no detectable LVOTgradient.Case No. 2A 51-year-old white male with ESLD second-ary to alcoholism and hepatitis C infection was en-rolled on the liver transplantation waiting list afterhe quit drinking. During his pre-transplant evalua-tion process, he noted chest tightness, dyspnea onexertion and near syncope. On examination, he wasjaundiced and had evidence of JVD. His cardiacexam was relevant for a grade II/VI systolic ejec-tion murmur with a diastolic component and a pos-itive fourth heart sound. The lungs were clear, andhe had mild ascites with 1+ pitting oedema of theankles and feet. Electrocardiography showed non-specific T wave changes. TTE revealed asymmetricleft ventricular hypertrophy (LVH), with IVS of21 mm, LVPW of 13 mm, a resting LVOT obstruc-tion with a peak gradient of 64 mm Hg at rest and102 mm Hg with provocation, mild SAM of anteriormitral leaflet (Fig. 2) and moderate mitral incom-petence with a normal LVEF, all consistent withHOCM. Cardiac catheterization documented mid-cavity obliteration with an LV-Ao gradient of120 mm during sinus rhythm and 220 mm Hg post-PVC,as well as grade II mitral regurgitation (Fig. 3).Figure 1. Assessment of left ventricular outflow gradient of patient 1 with hypertrophic cardiomyopathy before (A)and after (B) alcohol septal ablation using cardiac catheterization. Significant improvement of the gradient is visuali-zed (B). Left ventricular pressure tracing in grey colour and aortic pressure tracing in white. The arrow in A and Bdenotes post premature ventricular contraction pressure gradients.76Cardiology Journal 2008, Vol. 15, No. 1www.cardiologyjournal.orgHe continued to be symptomatic despite treatmentwith beta-blockers. He was deemed high risk forliver transplantation due to his symptomatic HOCMand high risk for surgical myomectomy due to hisESLD. Under TTE and fluoroscopy guidance, 4 mlof 100% ethanol was injected into the first septalartery. The LV-Ao gradient fell to 3 mm Hg postablation (Fig. 3). The patient had a good recoverywith improvement of his dyspnea and chest pain.Repeat echocardiography six months later showedan improvement of the LVOT resting gradient at27 mm Hg and amelioration of both IVS and LVPWdiameters (15 mm and 13 mm, respectively) withno signs of SAM of the anterior mitral leaflet. Thepatient underwent orthotopic liver transplantationin November of 2005 without major complications.One year after his transplant the patient continuedto report no dyspnea or angina even with exertion.DiscussionHypertrophic cardiomyopathy is a complexgenetic cardiac disease, diagnosed principally withTTE by demonstrating LVH, wall thickness of atleast 15 mm in adults, typically asymmetric, in theabsence of another cardiac or systemic disease ca-pable of producing a similar degree of hypertrophy(e.g., hypertension or aortic stenosis) [7]. HOCMrefers to the presence of a dynamic LVOT obstruc-tion usually caused by SAM of the anterior mitralvalve leaflet and projection of the hypertrophiedinterventricular septum into the outflow tract insystole. Management is aimed at alleviating symp-toms of heart failure, angina and syncope on onehand, and preventing sudden death in high-risk in-dividuals on the other. Medical therapy is the firstline of treatment and consists of beta-blockers, cal-cium channel antagonists and disopyramide, solelyor in combination. Surgical septal myomectomy ormore recently ASA are recommended for the smallsubgroup of patients (5%) who have both a largeoutflow gradient (usually > 50 mm Hg at rest or onprovocation) and moderate to severe heart failureor angina symptoms unresponsive to maximal med-ical therapy [7]. Historically, surgical septal myo-mectomy has been the “gold standard” treatmentfor HOCM patients refractory to medical therapy,perhaps due to more than 40 years of experience,a relatively low perioperative mortality rate (1–3%),high success rate with clinical improvement report-ed in up to 90% of patients, and excellent long-termsurvival [7, 8]. ASA emerged in 1995 [9] as an al-ternative procedure to surgery and has since grownquickly in popularity to the point that it is estimat-ed that more ASAs have been performed since itsintroduction than the total number of surgical myo-mectomies performed during the last 45 years [8].Studies have shown that ASA is as effective as sur-gery in improving symptoms and reducing LVOTgradient in the short- and intermediate-term; how-ever, there is still a lack of data regarding long-termfollow up, which is the main argument of its detrac-tors [10]. At present, there is no general agreementbetween cardiologists and surgeons regardingwhich procedure should be the first-line interven-tion for most HOCM patients after medical treat-ment failure. Patients with advanced age or sig-nificant comorbidities and/or relative contraindi-cations to surgery are often considered goodcandidates for ASA.ESLD patients undergoing general anesthesiaand non-hepatic surgery are at increased risk forperioperative complications, with an overall 30-dayFigure 2. Transthoracic echocardiography of patient 2with hypertrophic cardiomyopathy before alcohol sep-tal ablation. A. 5-chamber view showing hypertrophiedinterventricular septum and systolic anterior motion(SAM) of the anterior mitral valve leaflet (white arrow);B. M-mode across the mitral valve showing SAM of theanterior mitral valve leaflet (black arrow).77Fadi G. Hage et al., Hypertrophic obstructive cardiomyopathy and liver transplantswww.cardiologyjournal.orgmortality rate ranging between 11% and 17% [11–13].The surgical prognosis correlates with the severi-ty of cirrhosis. In one study, patients with Childclass A, B and C had 1-month mortality rates of3.6%, 31.7% and 54.5%, respectively [11]. The clin-ical outcomes are even poorer after cardiac surgeryin patients with ESLD, with mortality rates of 50%to 80% in class B patients [2, 4, 5, 11] and 100% inclass C patients [2, 5]. The difference in mortalitybetween cardiac and non-cardiac surgery in patientswith ESLD stems from the use of cardiopulmonarybypass, a known trigger for the production and re-lease of several vasoactive substances and cytotoxicchemicals that are responsible for much of the mor-bidity associated with open heart surgery [14, 15].These findings have been verified by the very re-cent report by Filsoufi et al. [16] in which opera-tive and 1-year mortality as well as postoperativecomplications were directly related to the Childclass in cirrhotic patients who underwent cardiacsurgery, and, significantly, no operative mortalityoccurred in patients who had cardiac surgery with-out the use of cardiopulmonary bypass.When this prognostic data is considered, it be-comes apparent that patients with cirrhosis, whosediseases are advanced enough for them to be consid-ered as liver transplant candidates, are absolute non-candidates for surgical septal myomectomy. ASA hasevolved as a very attractive treatment modality in thishigh-risk surgical group. However, it still remainsuncertain whether patients with HOCM should un-dergo ASA prior to or after liver transplantation.On the other hand, little is known about the riskof general anesthesia and major non-cardiac surgeryin patients with HOCM. In this regard, the incidenceof adverse cardiovascular events such as heart failure,Figure 3. Assessment of left ventricular outflow gradient of patient 2 with hypertrophic cardiomyopathy before (A, B)and after (C, D) alcohol septal ablation using Doppler echocardiography (A, C) and cardiac catheterization (B, D).Significant improvement of the gradient is noted. Left ventricular pressure tracing in grey colour and aortic pressuretracing in white.78Cardiology Journal 2008, Vol. 15, No. 1www.cardiologyjournal.orgischemia and arrhythmias, varied from 16% to 44%in the studies reviewed [17–20], with a strikinglylow rate of postoperative death and myocardial inf-arction (near 0%) in HOCM patients undergoingnon-cardiac surgery [18–20]. Perioperative cardio-vascular compromise is the main concern whenperforming a major surgery, such as liver transplan-tation, on patients with HOCM. However, most ofthese complications are transitory and treatable,and studies failed to report increased postsurgicalmortality rates in these individuals. Moreover,three case reports demonstrated no perioperativecardiovascular events in HOCM patients receivingorthotopic liver transplantation, analogous to ourfirst case [21, 22]. The other option is to performASA prior to surgery in order to avoid these report-ed potential perioperative complications [17–20],with the possible downside of delaying surgery. It isstill uncertain whether improvement of the LVOTby ASA will translate into reduction of the aforemen-tioned perioperative adverse cardiovascular events.We report here on the first case, to the best ofour knowledge, of an orthotopic liver transplantrecipient undergoing ASA and the second [23] ofa cirrhotic patient requiring ASA prior to livertransplantation. Our first patient with cirrhosis andasymptomatic HOCM had an uneventful liver trans-plantation but required ASA 1.5 years post-trans-plant due to worsening symptoms. The second pa-tient had ASA performed 15 months before livertransplantation, which facilitated his enlistment onthe transplant list. In both scenarios, the clinicaloutcome was favourable and the decision to performASA was mainly driven by symptomatic HOCM.Currently, there is no strong evidence to supporteither approach.In conclusion, ASA in ESLD patients withHOCM is safe and effective. ASA can be performedas a bridge to liver transplantation in symptomaticpatients. Patients with asymptomatic HOCM cansafely undergo liver transplantation and deferringASA until symptom development.AcknowledgementsThe authors do not report any conflict ofinterest regarding this work.References1. Maron MS, Olivotto I, Betocchi S et al. Effect of leftventricular outflow tract obstruction on clinical out-come in hypertrophic cardiomyopathy. New Engl JMed, 2003; 348: 295–303.2. Hayashida N, Shoujima T, Teshima H et al. Clinicaloutcome after cardiac operations in patients with cir-rhosis. Ann Thor Surg, 2004; 77: 500–505.3. Kaplan M, Cimen S, Kut MS, Demirtas MM. Cardiacoperations for patients with chronic liver disease.Heart Surg Forum, 2002; 5: 60–65.4. Klemperer JD, Ko W, Krieger KH et al. Cardiacoperations in patients with cirrhosis. Ann Thor Surg,1998; 65: 85–87.5. Suman A, Barnes DS, Zein NN, Levinthal GN,Connor JT, Carey WD. Predicting outcome after car-diac surgery in patients with cirrhosis: A comparisonof Child-Pugh and meld scores. Clin GastroenterolHepatol, 2004; 2: 719–723.6. Lopez PM, Martin P. Update on liver transplanta-tion: Indications, organ allocation, and long-termcare. Mount Sinai J Med New York, 2006; 73: 1056––1066.7. Maron BJ, McKenna WJ, Danielson GK et al. AmericanCollege of Cardiology/European Society of Cardiologyclinical expert consensus document on hypertrophiccardiomyopathy. 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Hypertrophic obstructive cardiomyopathy in liver transplant patients

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Hypertrophic obstructive cardiomyopathy in liver transplant patients

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