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Rhythm Disorders

Worsening Cardiomyopathy Despite Biventricular Pacing

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA Cardiology

Published Online: August 11, 2021

JAMA Cardiology Clinical Challenge

Article Information and Disclosures

Gustavo S. Guandalini, MD¹;

Matthew C. Hyman, MD, PhD¹

Author Affiliations

¹Section of Cardiac Electrophysiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania

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A man in his early 70s with a history of permanent atrial fibrillation treated with atrioventricular junction ablation and biventricular pacemaker implantation was hospitalized with decompensated heart failure and acute kidney injury. His history was notable for nonischemic cardiomyopathy with ejection fraction of 40% and idiopathic pulmonary fibrosis, for which he underwent single-lung transplant 9 years prior. Following diuresis and medical optimization, repeated echocardiogram showed a newly depressed ejection fraction (30%). His electrocardiogram (ECG) was notable for alternating QRS morphologies (Figure 1A). The left ventricular (LV) pacing vector was programmed from LV tip to right ventricular (RV) lead ring. During pacemaker threshold testing, a change in QRS morphology was observed as the output was reduced (Figure 1B). Chest radiography revealed severe right pulmonary fibrosis leading to ipsilateral mediastinal shift and pacemaker lead position unchanged from implantation.

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A man in his early 70s with a history of permanent atrial fibrillation treated with atrioventricular junction ablation and biventricular pacemaker implantation was hospitalized with decompensated heart failure and acute kidney injury. His history was notable for nonischemic cardiomyopathy with ejection fraction of 40% and idiopathic pulmonary fibrosis, for which he underwent single-lung transplant 9 years prior. Following diuresis and medical optimization, repeated echocardiogram showed a newly depressed ejection fraction (30%). His electrocardiogram (ECG) was notable for alternating QRS morphologies (Figure 1A). The left ventricular (LV) pacing vector was programmed from LV tip to right ventricular (RV) lead ring. During pacemaker threshold testing, a change in QRS morphology was observed as the output was reduced (Figure 1B). Chest radiography revealed severe right pulmonary fibrosis leading to ipsilateral mediastinal shift and pacemaker lead position unchanged from implantation.

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Article Information

Corresponding Author: Matthew C. Hyman, MD, PhD, Division of Cardiovascular Medicine, Electrophysiology Section, Hospital of the University of Pennsylvania, 3400 Spruce St, 9 Gates Pavilion, Philadelphia, PA 19104 (Matthew.Hyman@pennmedicine.upenn.edu).

Published Online: August 11, 2021. doi:10.1001/jamacardio.2021.2755

Conflict of Interest Disclosures: None reported.

Funding/Support: This work was supported by a grant from the Fondation Leducq (TNE FANTASY 19CV03).

Role of the Funder/Sponsor: The funder had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: We thank the patient’s daughter for granting permission to publish this information on the patient’s behalf.

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.