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Claim free CME just for enjoying this episode! Disclosures: None Jump to: Patient summary - Case media - Case teaching - References CardioNerds Case Reports PageCardioNerds Episode PageCardioNerds AcademyCardionerds Healy Honor Roll CardioNerds Journal ClubSubscribe to The Heartbeat Newsletter!Check out CardioNerds SWAG!Become a CardioNerds Patron! Patient Summary- Syncope and Pulmonary Hypertension A Somali woman in her mid-30s with no significant past medical history presented with shortness of breath and exertional syncope. EKG revealed evidence of RV strain. CTA-PE protocol did not show PE. However, there was RV dilation and subsequent echocardiogram demonstrated normal LV, but moderately reduced RV function with evidence of RV pressure and volume overload. RVSP was estimated to be 188 mmHg! Case Media ABCDEFGHClick to Enlarge A. CXR, B. ECG, C. PA measurements: Main PA measures 2.4 cm, right PA measures 2.3 cm, left PA measures 1.9 cm, D. Tricuspid valve Doppler, E. RA tracing, F. RV tracing, G. PA tracing, H. Wedge tracing CTA PE: No PE, markedly dilated pulmonary trunk at 4.7 cm. Right main pulmonary artery measures 3.1 cm. TTE: Parasternal long axis: Moderate right ventricular dilation compressing left ventricle. Global right ventricular function is moderately reduced. TTE: Parasternal long axis- RV view: Right ventricular dilation with mild pulmonary regurgitation TTE: Mild pulmonary regurgitation with dilation of main PA TTE: Paradoxical septal motion consistent with right ventricular pressure and volume overload. TTE: Apical 4 chamberParadoxical septal motion consistent with right ventricular pressure and volume overload. Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement. Paradoxical septal motion consistent with right ventricular pressure and volume overload.Moderate right ventricular dilation.Global right ventricular function is moderately reduced.Severe right atrial enlargement.Moderate to severe tricuspid regurgitation. TTE: Positive bubble study Episode Teaching Pearls Pulmonary hypertension (PH) can generally be categorized as pre-, post-, or combined pre- and post-capillary PH. Isolated pre-capillary pulmonary hypertension is characterized by: mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, a pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and a pulmonary vascular resistance (PVR) ≥ 3 Woods units (WU). Pulmonary arterial hypertension (PAH) (WHO Group 1) falls under pre-capillary pulmonary hypertension.Schistosomiasis is the most common cause of PAH (WHO Group I) worldwide. Approximately 7% of patient with hepatosplenic schistosomiasis have PAH. Some studies suggest that treatment of with praziquantel reverses vascular remodeling; however, there is point of no return, beyond which, anthelmintic therapies are ineffective to prevent progression.Exertional syncope and pericardial effusion are both risk factors for higher mortality in PAH.Women with severe PAH have extremely high risk of maternal morbidity and mortality. Endothelin receptor antagonists are contraindicated in pregnancy due to teratogenicity. Therefore, a pregnancy test must be obtained monthly while on this therapy.Patients with a lower socioeconomic status, based on median household income, have more advanced PAH at the time of diagnosis. Notes 1. How do you approach syncope? Syncope is a sudden transient loss of consciousness associated with absence of postural tone followed by complete and usually rapid recovery. There should be not be clinical evidence of “non-syncope” conditions including seizures, hypoglycemia, drug or alcohol intoxication, concussion due to head trauma and so forth. One approach to determining the etiology of the syncope is to consider 4 major categories: orthostatic, reflex-mediated, cardiac-obstructive,