SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Pulmonary hypertension (PH) is a rare initial presentation of multiple myeloma (MM). Herein, we report an unusual case of reversible PH with improved right heart failure following Rx of MM.
CASE PRESENTATION: A 42-year-old man presented with SOB and abdominal discomfort for the past 6 months, but worsened 3 days ago. 2 months ago, he suffered from fracture of the right 5th rib without preceding trauma. Exam: mildly pale, JVD, prominent P2 with S3 gallop heard best at LLSB, distended abdomen, 2+ pitting edema. TTE revealed normal sized LV with paradox motion of the IVS. The RV was also dilated. A high-velocity TR jet was detected with a systolic pulmonary pressure of 85 mmHg. The IVC was dilated (35 mm) without respiratory-related changes. Labs: normochromic anemia (Hb 9.6 g/dl), ↓ plt (120,000 /mm3), ↑ plasma protein concentration (9.8 g/dl). Skeletal X-ray showed multiple advanced lytic bone lesions. BM biopsy revealed 85% clonal plasma cells. According to the Durie and Salmon staging system, a diagnosis of MM stage IIIa with concomitant PH and right heart failure due to plasma cell disease was made. He received vincristine, adriamycin, dexamethasone (VAD) for Rx of MM. After 1 cycle, he was clinically improved. Hb ↑ (12.0 g/dL), plt count was normal. Right heart cath performed at 3 weeks after the start of Rx showed significant ↓ pulmonary artery pressure (PAP) (45/22 mmHg, mean 34 mmHg). TTE post-chemotherapy showed a ↓ RV size, trivial TR and disappearance of paradox motion of the IVS. The size of the IVC was within nml limits. LV function was normal (EF > 55%). Repeated BM biopsy showed 30% of plasma cells after the forth cycle of VAD. He remains in complete remission at 26-month follow-up after 2 successful stem cell transplantations.
DISCUSSION: PH is a rare initial presentation of MM. Small-vessel thrombosis as a result of plt activation at the site of endothelial injury has been implicated in the mechanism of PH.1 Amyloid deposition into pulmonary arteries may contribute to PH as the arterial amyloid deposition is common in AL amyloidosis.2 However, in our patient, we hypothesize that inflammatory cytokines related to myeloma cells likely play a pivotal role in ↑ pulmonary vascular resistance as evident by markedly ↓ systolic PAP by 40 mmHg in 3 weeks and improvement of right heart failure following Rx with chemotherapy and steroids.
CONCLUSIONS: This is the first case report of PH as an initial presentation of MM with potential reversibility following its Rx without involving modern therapies of PH such as endothelin antagonists and PDE-5 inhibitors.
Reference #1: 1. Hughes R, Tong J, Oates C, et al. Evidence for systemic endothelial dysfunction in patients and first-order relatives with pulmonary arterial hypertension. Chest 2005;128 6 Suppl:617.
Reference #2: 2. Lehtonena J, Kettunenb P, et al. Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma. International Journal of Cardiology 2007;115(1):29-30.
DISCLOSURE: The following authors have nothing to disclose: Pongpeera Taytawat
No Product/Research Disclosure Information