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Pulmonary Hypertension Case Study

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Pulmonary Hypertension (PH) is a rare and potentially fatal complication of hematopoietic stem cell transplantation (HSCT). PH arises from increased pulmonary vascular resistance leading to increased right ventricular pressure, right heart failure, and death. PH is often difficult to diagnose as symptoms can be nonspecific, including shortness of breath, fatigue, dizziness, weakness, and hypoxemia, and may also result in death if left untreated.1, 2
Bronchiolitis obliterans (BO) is a significant cause of morbidity and mortality in post-lung transplant and HSCT patients3. BO is a non-reversible obstructive lung disease in which bronchioles are compressed and narrowed by fibrosis and/or inflammation. This compression leads to persistent hypoxia. …show more content…

Although BO may be causative of PH, the treatment strategies for each are vastly different. We believe this case series may provide insight into PH and BO and act as a bridge to further study of possible treatment options.
Methods
We conducted a retrospective review of 291 consecutive allogeneic HSCT patients treated at Cincinnati Children’s Hospital Medical Center (CCHMC) from January 2009 to
December 2012. Clinical observations of patients’ symptoms at the time of diagnosis included shortness of breath, fatigue, dizziness, weakness, and hypoxemia. This research was performed with the approval of the institutional review board.
All patients with a clinical diagnosis of BO were identified, clinical records reviewed, and data extracted. Data collected included patient demographics, laboratory data, results of radiologic studies, pathology results, photographs of skin lesions, medical history, physical examination findings, treatment plan, and treatment outcome. All patients diagnosed with BO met standard diagnostic criteria for BO, including evidence of air trapping on high-resolution computed tomography …show more content…

Four patients (1.4%) were diagnosed with BO (Table 1). The median patient age was
7.9 years (range, 0.6 to 17.4), and 2 of the patients (50%) diagnosed with BO were male. Three received bone marrow grafts (75%), and the other graft was peripheral blood stem cells. BO was diagnosed in the four patients at a median of 196 days (range
131-305 days) after HSCT. Three of the four patients (75%) were diagnosed with PH after the diagnosis of BO at a median of 46 days (range 2-89 days). The three patients diagnosed with PH and BO were administered myeloablative conditioning regimens, while the one patient without PH was administered a reduced intensity conditioning regimen. All four patients had a history of graft-versus-host disease (GVHD). (Table 1)
The diagnosis of BO was made in all 4 patients by high-resolution CT demonstrating air trapping. Two patients received pulmonary function tests (PFT) showing decreased forced expiratory volume in one second (FEV1). PFTs were not completed in the

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