Background:
Acromegaly is associated with cardiac complications in over 50% of patients. Though diastolic dysfunction, biventricular hypertrophy, and arrhythmias are common, progression to systolic dysfunction and overt heart failure is rare. Here we present a case of acromegalic cardiomyopathy culminating in cardiogenic shock (CS), mechanical circulatory support (MCS), and orthotopic heart transplantation (OHT).
Case:
A 39-year-old man with a history of type 2 diabetes initially presented with dyspnea at age 35 and was found to have a dilated cardiomyopathy with LVEF 15–20% and a nonischemic pattern on cardiac MRI. Left heart catheterization was negative for CAD, and genetic testing was unrevealing. He was diagnosed with nonischemic cardiomyopathy (NICM), started on guideline-directed medical therapy (GDMT), and received an ICD.
Four years later, he presented to an outside hospital with CS. Right heart catheterization revealed a CI of 1.6, RA pressure of 15 mmHg, PAP 57/35 mmHg, and PCWP of 31 mmHg. He was initiated on milrinone and transferred to our cardiac intensive care unit for advanced management.
Decision-making:
At our center, physical exam revealed features suggestive of acromegaly. Laboratory workup showed IGF-1 of 870 ng/mL and GH >65 ng/ml. Pituitary imaging identified a 2.5 cm macroadenoma.
Despite escalating inotropes, vasopressors, and intra-aortic balloon pump (IABP) support, he had progressive CS. He was transitioned to VA-ECMO with left ventricular unloading via Impella CP.
Multidisciplinary evaluation determined that he was not a candidate for durable LVAD due to severe biventricular dysfunction, ongoing vasopressor requirement, and uncertain reversibility of acromegalic cardiomyopathy. Given continued hemodynamic instability despite maximal temporary support, he was listed for urgent orthotopic heart transplant (OHT). After 8 days on ECPELLA, he successfully underwent OHT.
Post-transplant, he initially required inhaled nitric oxide for elevated pulmonary pressures, vasopressor support, and renal replacement therapy. He demonstrated gradual recovery, with successful weaning of vasopressors and ventilatory support, followed by extubation and rehabilitation. Serial right heart catheterizations showed improving graft function.
He was initiated on octreotide and later underwent MRI and neurosurgical evaluation, with transsphenoidal resection of the pituitary macroadenoma planned as definitive therapy.
Discussion:
This case illustrates the trajectory from undiagnosed acromegaly to advanced heart failure and highlights the complexity of managing CS in a patient with active hormonal cardiotoxicity. Literature suggests GH control can improve cardiac parameters and stabilize heart failure in early disease,1 but data are limited in cases of end-stage heart failure. Our case demonstrates that despite the high-risk hormonal milieu, OHT remains a viable option when comprehensive multidisciplinary management is employed.
In particular, the timely escalation of MCS and early transfer to a tertiary cardiac ICU were crucial in bridging this patient to transplant. This case reinforces the critical role of intensivists and heart failure cardiologists in recognizing reversible endocrine cardiomyopathies and guiding advanced therapies, including MCS and transplant, in patients with acute decompensated heart failure due to rare etiologies.