- A 45-year-old woman with an established diagnosis of Loeys-Dietz syndrome presented to Massachusetts General Hospital with class IV heart failure secondary to ischemic cardiomyopathy
- She was listed for cardiac transplantation and a suitable organ became available, and then underwent heart transplantation
- At one year post-transplantation, the patient is doing well on a standard immunosuppressive regimen and has a normal ejection fraction
- Physicians should not hesitate to list patients with connective tissue disorders for heart transplantation
Loeys-Dietz syndrome (LDS) is a connective tissue disorder similar to Marfan syndrome but with more severe cardiovascular manifestations. Heart transplantation can be particularly difficult in patients with connective tissue disorders due to the fragility of the aorta and surrounding vascular structures.
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In The Annals of Thoracic Surgery, Amy G. Fiedler, MD, cardiac surgery fellow, and David A. D'Alessandro, MD, surgical director of Heart Transplantation and Ventricular Assist Devices, report the first successful heart transplantation in an individual with LDS.
At presentation, the 45-year-old female patient had an established diagnosis of LDS. Her history included atrial septal defect repair, stroke and myocardial infarction after spontaneous left main coronary artery dissection. Following the myocardial infarction, she had undergone coronary artery bypass surgery.
Despite revascularization, the patient had developed New York Heart Association class IV heart failure secondary to ischemic cardiomyopathy, and she was being maintained on home inotropic therapy. She had experienced a persistent decline in the ability to perform activities of daily living and required frequent hospitalizations.
At the time the patient presented to Massachusetts General Hospital, her ejection fraction was 29%. The multidisciplinary cardiac transplantation team listed her as status 1A due to her dependence on inotropic support.
Heart Transplantation and Follow-up
The patient underwent heart transplantation in July 2017 when a suitable donor organ became available. Her ascending aorta was thin and friable, so the surgeons dispensed with their usual procedure of ascending aorta cannulation and chose to cannulate the femoral artery directly with bicaval venous cannulation. They modified their aortic anastomotic technique by using a strip of bovine pericardium to reinforce the suture line.
Cardiopulmonary bypass time was 206 minutes and the patient was separated from cardiopulmonary bypass without incident. Her hospitalization was uncomplicated. She underwent a routine cardiac biopsy on postoperative day seven without evidence of rejection, and she was discharged to home on day 10.
Now, at one-year post-transplant, the patient is doing well on a standard immunosuppression regimen. She has not experienced any additional spontaneous vascular dissections, and the standard follow-up protocol has demonstrated normal ejection fraction, no structural abnormalities and no aneurysmal dilation along the aortic or pulmonary artery suture lines.
Advice for Physicians
Dr. Fiedler and Dr. D'Alessandro urge physicians not to hesitate to list patients with connective tissue disorders for heart transplantation. Ten-year survival after heart transplantation is known to be similar for patients with and without Marfan syndrome, about 50% to 60%. The first successful heart transplantation in a patient with Ehlers-Danlos syndrome was performed in 2015 without postoperative complications.
For patients with LDS, the authors caution, heart transplant teams should be prepared to use alternative arterial cannulation strategies and to reinforce or replace the aorta, which may require circulatory arrest.
Following heart transplantation, medical management of a patient with LDS should include an angiotensin receptor blockade for life. Patients should be screened with magnetic resonance angiography or computed tomography angiography at baseline and every 12 to 24 months to assess for aneurysms throughout the arterial tree.
Refer a patient to the Mass General Heart Center
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