Sleep-Related Hypoxia, Right Ventricular Dysfunction, and Survival in Patients With Group 1 Pulmonary Arterial Hypertension.

BACKGROUND: Group 1 pulmonary arterial hypertension (PAH) is a progressive fatal condition characterized by right ventricular (RV) failure with worse outcomes in connective tissue disease (CTD). Obstructive sleep apnea and sleep-related hypoxia may contribute to RV dysfunction, though the relationship remains unclear. OBJECTIVES: The aim of this study was to prospectively evaluate the association of the apnea-hypopnea index (AHI) and sleep-related hypoxia with RV function and survival. METHODS: Pulmonary Vascular Disease Phenomics (National Heart, Lung, and Blood Institute) cohort participants (patients with group 1 PAH, comparators, and healthy control participants) with sleep studies were included. Multimodal RV functional measures were examined in association with AHI and percentage of recording time with oxygen saturation <90% (T90) per 10-unit increment. Linear models, adjusted for demographics, oxygen, diffusing capacity of the lungs for carbon monoxide, pulmonary hypertension medications, assessed AHI and T90, and RV measures. Log-rank test/Cox proportional hazards models adjusted for demographics, oxygen, and positive airway pressure were constructed for transplantation-free survival analyses. RESULTS: Analysis included 186 participants with group 1 PAH with a mean age of 52.6 ± 14.1 years; 71.5% were women, 80.8% were Caucasian, and there were 43 events (transplantation or death). AHI and T90 were associated with decreased RV ejection fraction (on magnetic resonance imaging), by 2.18% (-2.18; 95% CI: -4.00 to -0.36; P = 0.019) and 0.93% (-0.93; 95% CI: -1.47 to -0.40; P < 0.001), respectively. T90 was associated with increased RV systolic pressure (on echocardiography), by 2.52 mm Hg (2.52; 95% CI: 1.61 to 3.43; P < 0.001); increased mean pulmonary artery pressure (on right heart catheterization), by 0.27 mm Hg (0.27; 95% CI: 0.05 to 0.49; P = 0.019); and RV hypertrophy (on electrocardiography), 1.24 mm (1.24; 95% CI: 1.10 to 1.40; P < 0.001). T90, but not AHI, was associated with a 17% increased 5-year risk for transplantation or death (HR: 1.17; 95% CI: 1.07 to 1.28). In non-CTD-associated PAH, T90 was associated with a 21% increased risk for transplantation or death (HR: 1.21; 95% CI: 1.08 to 1.34). In CTD-associated PAH, T90 was associated with RV dysfunction, but not death or transplantation. CONCLUSIONS: Sleep-related hypoxia was more strongly associated than AHI with measures of RV dysfunction, death, or transplantation overall and in group 1 non-CTD-associated PAH but only with RV dysfunction in CTD-associated PAH. (Pulmonary Vascular Disease Phenomics Program [PVDOMICS]; NCT02980887).

A 48-Year-Old Athletic Man With Bradycardia During Sleep.

CASE PRESENTATION: A 48-year-old athletic man with history of dyslipidemia and second-degree atrioventricular block (Mobitz type I 2:1 atrioventricular block) endorses sleep-related difficulties at his annual physical examination. Mobitz type I block was diagnosed 11 years earlier when the patient presented to his primary care physician with left-sided chest pain for 2 months. He was evaluated by cardiology, with a normal stress echocardiogram. The impression was that the chest pain was nonanginal. He describes sleeping for only a few hours at a time and has nighttime awakenings. He does not wake up feeling refreshed. He experiences passive daytime sleepiness and evening somnolence. He is very active, runs every day, and has participated in several half-marathons. He currently undergoes high-intensity interval training, including running and lifting. Per his wife, the patient snores loudly and has apneic episodes during sleep. One of his siblings has OSA. He denies excessive sleepiness, with an Epworth Sleepiness Scale score of 2.

Emphysematous Pyelonephritis Associated With Emphysematous Gastritis and Air in the Portal Vein.

Emphysematous gastritis with portal venous air is a rare condition usually caused by gas forming organisms. This may be secondary to local spread of an infection through the mucosa or rarely hematogenous dissemination from a distant focus. We present a young diabetic woman with uncontrolled diabetes mellitus who was admitted with sepsis and severe abdominal symptoms. Investigation revealed emphysematous pyelonephritis due to E. coli infection associated with emphysematous gastritis and air in the portal tract. She improved with broad spectrum antibiotics, fluid resuscitation and electrolyte and diabetic management. To our knowledge this is the first report showing the association between emphysematous pyelonephritis and gastritis with air in the portal system.