Dasatinib and other tyrosine kinase inhibitors are commonly utilized in the management of chronic myelogenous leukemia. Pulmonary hypertension is an important adverse event associated with dasatinib. Mechanisms for pulmonary hypertension include pulmonary endothelial injury, apoptosis, and increased susceptibility to other triggers for pulmonary hypertension. The diagnosis is suspected based on symptoms, suggested by echocardiographic findings, and confirmed with right heart catheterization. Management includes discontinuation of dasatinib and initiation of pulmonary vasodilators. Persistent pulmonary hypertension is present in up to one third of patients after cessation of dasatinib. Other tyrosine kinase inhibitors, including bosutinib, lapatinib, and ponatinib have also been implicated in pulmonary hypertension in small series, although evidence for causation is less robust. A high index of suspicion, continued vigilance for pulmonary hypertension with long-term use, and early therapy are important in optimizing outcomes in this population.
Background
Syncope has many aetiologies but from a cardiac standpoint, if arrhythmogenic and ischaemic causes are not present, obstructive lesions should be considered. Cardiac spindle cell sarcomas are incredibly rare and difficult to cure.
Case summary
A 62-year-old man presented for exercise stress test and had a syncopal episode on the treadmill. He was found to have a massive mass obstructing the transmitral flow. Patient was taken to the operating room and the mass was resected successfully. Histopathological confirmation revealed the mass to be a cardiac intimal sarcoma. Patient was initiated on a trial regimen of doxorubin, ifosfamide, and mesna.
Discussion
Cardiac intimal sarcomas are aggressive cancers and are difficult to treat; there are no established treatment guidelines. They can lead to obstruction of blood flow through the cardiac chambers. From a cardiac perspective, without arrhythmogenic and ischaemic causes of syncope, obstructive lesions should be considered.
Right heart failure (RHF) after left ventricular assist device (LVAD) is associated with poor outcomes. Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) defines RHF as elevated right atrial pressure (RAP) plus venous congestion. The purpose of this study was to examine the diagnostic performance of the noninvasive INTERMACS criteria using RAP as the gold standard. We analyzed 108 patients with LVAD who underwent 341 right heart catheterizations (RHC) between January 1, 2006, and December 31, 2013. Physical exam, echocardiography, and laboratory data at the time of RHC were collected. Conventional two-by-two tables were used and missing data were excluded. The noninvasive INTERMACS definition of RHF is 32% sensitive (95% CI, 0.21-0.44) and 97% specific (95% CI, 0.95-0.99) for identifying elevated RAP. Clinical assessment failed to identify two-thirds of LVAD patients with RAP > 16 mm Hg. More than half of patients with elevated RAP did not have venous congestion, which may represent a physiologic opportunity to mitigate the progression of disease before end-organ damage occurs. One-quarter of patients who met the noninvasive definition of RHF did not actually have elevated RAP, potentially exposing patients to unnecessary therapies. In practice, if any component of the INTERMACS definition is present or equivocal, our data suggest RHC is warranted to establish the diagnosis.
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