Application of CRT-D in a Marfan syndrome patient with chronic heart failure accompanied by ventricular tachycardia and ventricular fibrillation

Application of CRT-D in a Marfan syndrome patient with chronic heart failure accompanied by ventricular tachycardia and ventricular fibrillation 1 first-author †E-mail:1339220837@qq.comhjyuo@163.com †E-mail:1339220837@qq.comhjyuo@163.com 1339220837@qq.com 1 1 1 1 1 2 1 corresp ‡Corresponding Author ‡Corresponding Author †E-mail:1339220837@qq.comhjyuo@163.com †E-mail:1339220837@qq.comhjyuo@163.com 1339220837@qq.com Department of Cardiology, Hangzhou First People’s Hospital, Hangzhou 310006, China Department of Cardiology, Hangzhou First People’s Hospital, Hangzhou 310006, China Department of Cardiac Ultrasound, Hangzhou First People’s Hospital, Hangzhou 310006, China Department of Cardiac Ultrasound, Hangzhou First People’s Hospital, Hangzhou 310006, China Marfan syndrome is a systemic connective tissue disease that could affect the cardiovascular system and eventually lead to heart enlargement and heart failure with high mortality, mainly due to progressive heart failure and/or sudden cardiac death caused by malignant arrhythmia. Here we report that a patient received a cardiac resynchronization therapy-defibrillator (CRT-D) with a pre-monitor function for heart failure and experienced obvious improvements in his cardiac function. Postoperative follow-up showed that the patient had reduced morbidity and hospitalization for heart failure, and also experienced improved quality of life. 1 A 69-year-old male patient was admitted six months ago due to coughing, and expectoration with a fever for four days. The patient was diagnosed with Marfan syndrome and accepted ascending aorta and had an aortic valve replacement surgery 14 years ago. With a 10-year history of hypertension and a cerebral infarction 5 years ago, he had been suffering repeated symptoms of heart failure during the past year, and was hospitalized after diagnosis of lung infection, worsening heart failure and atrial fibrillation. On the night of admission he went through sudden cardiac arrest of the ventricular fibrillation, and survived after immediate defibrillation and resuscitation procedures, with heart rhythm restored through atrial fibrillation. Physical examination revealed jugular vein filling, and diminished vesicular sounds on both sides of the chest. The heart rate was irregular at 70 beats/min (bpm). II/6 grade systolic murmur (SM) at mitral area and III/6 grade SM at aortic valve area were also heard. The heart was enlarged and no edema was found in either legs. A chest X-ray showed right lower lung infection and pleural effusion, with a cardiothoracic ratio of 0.65; B ultrasound showed a bilateral pleural effusion (median volume); echocardiography showed a left ventricular enlargement (left atrium 5.2 cm, left ventricular 6.4 cm) and a left ventricular dysfunction (ejection fraction (EF) 0.11); sequential electrocardiographs (ECGs) showed sinus rhythm with atrial fibrillation, complete left bundle branch block (CLBBB), and a left ventricular hypertrophy with strain. On the basis of inotropes, digoxin, lotensin, diuretics, amiodarone, warfarin, and a small dose of carvedilol as well as antibiotics, he received an implant INSYNC SENTRYTM7298 CRT-D (Bristow et al., 2004 2004 ). The parameters are listed in Table 1 1 . Due to the patient’s severe condition, a defibrillation threshold test (DFT) was not carried out during the procedure. The settings of the tachycardia diagnostic criteria were: ventricular tachycardia (VT), 130–150 bpm, monitor; fast ventricular tachycardia (FVT), 150–180 bpm, antitachycardia pacing (ATP); ventricular fibrillation (VF), > 182 bpm, shock. Lead Threshold (V) Impedance (Ω) Amplitude (mV) Left ventricle 0.5 769 17.9 Right ventricle 0.5 1280 14.2 Right atria Not tested (AF) 560 2.6 The patient still felt chest tightness and shortness of breath two days after the procedure, and complained of shock in the morning of the third day. The programmer showed 5 VF events, which led to 6 shock therapies (two shocks for one of the events), and all events were completed successfully. The patient continued to receive optimized drug therapy. Thereafter, an ECG showed atrial fibrillation and biventricular pacing, with QRS wave duration of 150 ms (Chung et al., 2008 2008 ). His sinus rhythm was restored after repeated defibrillation therapies due to recurrent ventricular tachycardia ventricular fibrillation. In the beginning, the sinus rhythm could only be sustained for quite short periods of time, but with administration of amiodarone and carvedilol, sustained atrial fibrillation gradually converted into paroxysmal atrial fibrillation, and finally converted into sustained sinus rhythm. Twenty days after the implantation of CRT-D, the patient felt well. One month after the implantation, parameter optimization was carried out guided by tissue Doppler imaging (TDI) (Barsheshet et al., 2011 2011 ). The left ventricular filling was evaluated by mitral time-velocity integral (TVI) during the diastolic phase (Dohi et al., 2005 2005 ), and mitral regurgitation was evaluated by TVI of the mitral regurgitation. The optimal atrioventricular (AV) interval was identified through an increased left ventricular filling and reduced mitral regurgitation, i.e., 100 ms for sensed atrioventricular (SAV) and 130 ms for paced atrioventricular (PAV). The time to peak myocardial systolic velocity at septum and lateral left ventricular wall was recorded from the TDI (Penicka et al., 2004 2004 ), and the difference between them was calculated to evaluate the desynchrony; the TVI of the aortic outflow was recorded as an indicator of left ventricular output (Mele et al., 2006 2006 ). The optimal left ventricular to right ventricular (VV) interval was defined as that which could best restore inter- and intra-ventricular synchrony. The test showed that when the VV interval (Baker et al., 2007 2007 ) was set to 32 ms, the difference between the time to peak myocardial systolic velocity at septum and the lateral left ventricular wall decreased to 63 ms from 176 ms before optimization (Fig. 1 1 ). (a) Difference between time to peak myocardial systolic velocity at septum and lateral left ventricular wall was 176 ms; (b) When the VV interval was set to 32 ms, the difference between the time to peak myocardial systolic velocity at septum and the lateral left ventricular wall was shortened to 63 ms Echocardiography parameters six months after implantation are shown in Table 2 2 . A built-in compass of CRT-D showed that the heart rate gradually declined and stabilized at 60 to 70 bpm with increases in the patient’s daily activities. The pre-monitor function of the heart failure in six months after the operation showed that transthoracic impedance gradually increased and stabilized, indicating significant improvement of the pulmonary edema. LAD: left atrial diameter; LVEDD: left ventricular end-diastolic diameter; LVEF: left ventricular ejection fraction; NYHA: New York Heart Association Group LAD (mm) LVEDD (mm) LVEF NYHA class QRS duration (s) Before 51.69 63.72 0.11 4 0.15 After 39.20 64.20 0.45 2–3 0.15 The patient was suffering a severe condition, which led to repeated malignant arrhythmia and defibrillation. Thus we proposed that timely implantation of CRT-D would bring more benefit for the patient despite a higher risk with these procedures. The efficacy of implantable cardioverter defibrillator (ICD) for primary and secondary prevention of sudden cardiac death (SCD) has been proven in a large number of randomized controlled trials. In some trials, DFT was carried out to ensure that ICD or CRT-D could appropriately cease ventricular arrhythmias. Nevertheless, Russo et al. ( 2005 2005 ) suggested that a simple application of a defibrillator with high output (≥35 J) in 48% of the study patients (3% of the entire installation ICD crowd) could not get the appropriate DFT, and 6% of ICD recipients had excessive DFT (safety margin < 10 J). This patient did not test intraoperative DFT due to a severely impaired cardiac function, but the defibrillation was successful with the highest energy to prevent SCD. There is no unified standard to decide which patient population should undergo a scheduled DFT, but it has been suggested that patients with a left ventricular diastolic diameter ≥80 mm or with severely impaired heart function should suspend the DFT to ensure their safety during the procedure. The patient was diagnosed as atrial fibrillation with obviously enlarged left atria, and was generally considered not necessary or feasible for cardioversion. For CRT candidates with permanent atrial fibrillation, it is generally accepted that atrioventricular node ablation is a good way to ensure dual-chamber pacing. This patient in our report initially suffered recurrent ventricular tachycardia, and sinus rhythm restored after defibrillation therapy could only be sustained for a quite short period of time. With administration of amiodarone and carvedilol, and improvement in cardiac function, sustained atrial fibrillation gradually converted into paroxysmal atrial fibrillation, and finally converted into sustained sinus rhythm 20 d after the implantation of CRT-D. This prompted us to take a review of the patients with heart failure and atrial fibrillation that were believed to be appropriate to receive cardioversion, and to evaluate the probability and necessity of these patients to go through medical and/or electrical cardioversion after CRT. Especially, if early restoration of sinus rhythm in these patients could help to maintain the atrioventricular synchrony, it would be more valuable for long-term prognosis improvement. This patient received CRT-D with pre-monitor function of heart failure, with the unique OptiVol™ technology that could accurately monitor the status of pulmonary edema during heart failure. For this patient, we found that the transthoracic impedance gradually reached steady status with improvement in cardiac function during follow-up. We believe that this would help patients and doctors to take timely measures to reduce morbidity and hospitalization for heart failure, and improve quality of life (Suffoletto et al., 2006 2006 ), and finally improve prognosis. Echocardiography is playing an increasingly important role in the study of patients with clinical and echocardiographic criteria for cardiac resynchronization therapy. The case presented highlights the importance of intraventricular dyssynchrony, identified by TDI. In this case through VV optimization, the difference between the time to peak myocardial systolic velocity at the septum and lateral left ventricular wall was shortened to 63 ms, which showed that the intraventricular dyssynchrony was improved and that the haemodynamics got better. From what we discussed above, we can draw the conclusion that CRT-D made sense for this patient. The patient after the synchronization, showed ventricular hemodynamic improvement resulting in improvement in the cardiac function. VF was effectively controlled to improve the arrhythmia, and the cardiac function was improved thereby. A strong adaptation of preoperative choice is also an important aspect for the patient. References JH Baker , J McKenzie , S Beau , GS Greer , J Porterfield , M Fedor , S Greenberg , EG Daoud , R Corbisiero , JR Bailey , 等 . Acute evaluation of programmer-guided AV/PV and VV delay optimization comparing an IEGM method and echocardiogram for cardiac resynchronization therapy in heart failure patients and dual-chamber ICD implants . J Cardiovasc Electrophysiol , 2007 . 18 ( 2 ): 185 - 191 . 17338767 DOI: 10.1111/j.1540-8167.2006.00671.x http://doi.org/10.1111/j.1540-8167.2006.00671.x . Acute evaluation of programmer-guided AV/PV and VV delay optimization comparing an IEGM method and echocardiogram for cardiac resynchronization therapy in heart failure patients and dual-chamber ICD implants A Barsheshet , I Goldenberg , AJ Moss , M Eldar , DT Huang , S McNitt , HU Klein , WJ Hall , MW Brown , JJ Goldberger , 等 . Response to preventive cardiac resynchronization therapy in patients with ischaemic and nonischaemic cardiomyopathy in MADIT-CRT . Eur Heart J , 2011 . 32 ( 13 ): 1622 - 1630 . 21075774 DOI: 10.1093/eurheartj/ehq407 http://doi.org/10.1093/eurheartj/ehq407 . Response to preventive cardiac resynchronization therapy in patients with ischaemic and nonischaemic cardiomyopathy in MADIT-CRT MR Bristow , LA Saxon , J Boehmer , S Krueger , DA Kass , T de Marco , P Carson , L DiCarlo , D DeMets , BG White , 等 . Cardiac-resynchronization therapy with or without an implantable defibrillator in advanced chronic heart failure . N Engl J Med , 2004 . 350 ( 21 ): 2140 - 2150 . 15152059 DOI: 10.1056/NEJMoa032423 http://doi.org/10.1056/NEJMoa032423 . Cardiac-resynchronization therapy with or without an implantable defibrillator in advanced chronic heart failure ES Chung , AR Leon , L Tavazzi , JP Sun , P Nihoyannopoulos , J Merlino , WT Abraham , S Ghio , C Leclercq , JJ Bax , 等 . Results of the predictors of response to CRT (PROSPECT) trial . Circulation , 2008 . 117 ( 20 ): 2608 - 2616 . 18458170 DOI: 10.1161/CIRCULATIONAHA.107.743120 http://doi.org/10.1161/CIRCULATIONAHA.107.743120 . Results of the predictors of response to CRT (PROSPECT) trial K Dohi , M Suffoletto , L Ganz , M Zenati , J Gorcsan . Utility of echocardiographic tissue synchronization imaging to redirect left ventricular lead placement for improved cardiac resynchronization therapy . Pacing Clin Electrophysiol , 2005 . 28 ( 5 ): 461 - 465 . 15869683 DOI: 10.1111/j.1540-8159.2005.40056.x http://doi.org/10.1111/j.1540-8159.2005.40056.x . Utility of echocardiographic tissue synchronization imaging to redirect left ventricular lead placement for improved cardiac resynchronization therapy D Mele , G Pasanisi , F Capasso , A de Simone , MA Morales , D Poggio , A Capucci , G Tabacchi , L Sallusti , R Ferrari . Left intraventricular myocardial deformation dyssynchrony identifies responders to cardiac resynchronization therapy in patients with heart failure . Eur Heart J , 2006 . 27 ( 9 ): 1070 - 1078 . 16574689 DOI: 10.1093/eurheartj/ehi814 http://doi.org/10.1093/eurheartj/ehi814 . Left intraventricular myocardial deformation dyssynchrony identifies responders to cardiac resynchronization therapy in patients with heart failure M Penicka , J Bartunek , B de Bruyne , M Vanderheyden , M Goethals , M de Zutter , P Brugada , P Geelen . Improvement of left ventricular function after cardiac resynchronization therapy is predicted by tissue Doppler imaging echocardiography . Circulation , 2004 . 109 ( 8 ): 978 - 983 . 14769701 DOI: 10.1161/01.CIR.0000116765.43251.D7 http://doi.org/10.1161/01.CIR.0000116765.43251.D7 . Improvement of left ventricular function after cardiac resynchronization therapy is predicted by tissue Doppler imaging echocardiography AM Russo , W Sauer , EP Gerstenfeld , HH Hsia , D Lin , JM Cooper , S Dixit , RJ Verdino , HM Nayak , DJ Callans , 等 . Defibrillation threshold testing: is it really necessary at the time of implantable cardioverter-defibrillator insertion? . Heart Rhythm , 2005 . 2 ( 5 ): 456 - 461 . 15840466 DOI: 10.1016/j.hrthm.2005.01.015 http://doi.org/10.1016/j.hrthm.2005.01.015 . Defibrillation threshold testing: is it really necessary at the time of implantable cardioverter-defibrillator insertion? MS Suffoletto , K Dohi , M Cannesson , S Saba , J Gorcsan . Novel speckle-tracking radial strain from routine black-and-white echocardiographic images to quantify dyssynchrony and predict response to cardiac resynchronization therapy . Circulation , 2006 . 113 ( 7 ): 960 - 968 . 16476850 DOI: 10.1161/CIRCULATIONAHA.105.571455 http://doi.org/10.1161/CIRCULATIONAHA.105.571455 . Novel speckle-tracking radial strain from routine black-and-white echocardiographic images to quantify dyssynchrony and predict response to cardiac resynchronization therapy Compliance with ethics guidelines Hai-ying XU, Yi-zhou XU, Feng LING, Zhong YU, Jun YANG, Xu DUAN, Bei WANG, and Jin-yu HUANG declare that they have no conflict of interest. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000(5). Informed consent was obtained from all patients for being included in the study. 10.1631/jzus.BQICC710 2013-07-03 2013-07-03 2013-07-20 2022-01-05 Copyright © Zhejiang University and Springer-Verlag Berlin Heidelberg 2013 2013 Journal of Zhejiang University-SCIENCE B (Biomedicine and Biotechnology) 08 14