Fournier's gangrene is rapidly progressive necrotizing fasciitis that mainly affects the male perineum. Despite the advancement in surgical intervention, Fournier's gangrene carries high rates of mortality. Here, we present a 51-year-old male with hypertension and history of alcohol abuse presented to the emergency department with scrotal pain and swelling for a one-week period without preceding trauma to perineal area. He underwent emergent surgical debridement for and extensive necrotizing fasciitis. Early initiation of antibiotics, surgical intervention and good wound care postoperatively were cornerstone in his recovery.

Hypertriglyceridemia-induced acute pancreatitis (HGAP) is the third most common etiology of acute pancreatitis. HGAP can be attributed to genetic disturbances in triglyceride metabolism or multiple secondary causes. Here, we presented three cases for HGAP and explored different therapeutic approaches for treating HGAP. A case series of three patients who presented with HGAP and underwent different therapeutic approaches was conducted. The first patient was a 37-year-old male who presented with nonsevere HGAP; he was treated with conservative therapy with insulin and heparin infusion, which resulted in clinical and laboratory improvement. The second patient was a 64-year-old male with human immunodeficiency virus on multiple highly active antiretroviral therapy. He presented with severe HGAP and multiorgan failure. After initiation of therapeutic plasma exchange, his HGAP resolved. The third patient was a 28-year-old male who presented with recurrent episodes of HGAP; his conservative therapy failed and was eventually escalated to therapeutic plasma exchange (TPE). HGAP can be attributed to genetic disturbances of lipid or secondary etiologies. A nonsevere form of HGAP can be managed with conventional therapy including insulin and heparin; however, severe HGAP may require TPE. K E Y W O R D S hypertriglyceridemia, pancreatitis, therapeutic plasma exchange

Anomalous pulmonary venous drainage (APVD) is a rare congenital disorder with pulmonary vein drainage into systemic circulation. We present a case of APVD with bilateral upper lobe venous drainage into the SVC and coexisting post capillary pulmonary hypertension. CASE PRESENTATION:A 72-year old gentleman with Down syndrome and cognitive impairment presented to pulmonary clinic for evaluation of pulmonary hypertension and dyspnea. It was difficult to discern by history, if reported symptoms were related to deconditioning or a result of dyspnea from cardiopulmonary disease. Exam was overall benign. However, 6-minute walk test was abnormal and revealed hypoxemia after 3 minutes, requiring 2 liters of supplemental oxygen by nasal cannula. Echocardiogram showed RVSP of 44 mmHg, enlarged RV with decreased LV internal cavity size and preserved ejection fraction. A contrast chest CT (Figure 1) showed partial APVD of the right upper lobe into the SVC and left upper lobe with APVD into the subclavian vein and into SVC (Figure 2). A right heart catheterization performed showed right atrial pressure of 16 mmHg, mean pulmonary artery pressure of 40 mmHg, wedge pressure of 20 mmHg, cardiac output of 7.5 L/min, pulmonary vascular resistance of 2.6 Wood units. Oxygen saturation showed IVC 76%, SVC 99%, RA 84%, RV 83%, PA 83%, PA wedge 87%. Shunt fraction (Qp:Qs) was calculated at 1.3. Furosemide was started to decrease preload. The patient was then referred to structural cardiology for repair of the defect due to progression of PH and RV dysfunction.DISCUSSION: Partial APVD, in contrast to total APVD, occurs when a proportion of pulmonary venous blood bypasses the left atrium, resulting in an abnormal shunt fraction. The prevalence of partial APVD is between 0.4 to 0.7%.1 A shunt fraction is abnormal when either it is greater than 1 (left to right shunt), or less than 1 (right to left shunt). In our patient, the failure of embryological tissue migration resulted in partial APVD, leading to increased systemic return to the RV, and over time resulting in RV dysfunction, potentially fatal. For this reason, it is important that the defect gets repaired to prevent future major cardiac event.CONCLUSIONS: While majority of literature report partial APVD to anomalous systemic circulation, to our knowledge this is the first reported case of bilateral partial APVD to an anatomically normal systemic circulation.

In patients with underlying lung disease, pulmonary evaluation is required prior to air travel due to hypoxic conditions in higher altitudes. We present a case of a young man aspiring to become a pilot, found to have a congenital cardiopulmonary defect of pulmonary atresia. CASE PRESENTATION: An 18 year old male with right pulmonary atresia and intact ventricular septum (PA-IVS) presented to the pulmonary clinic seeking medical clearance for flight training school, endorsing fatigue and dyspnea on exertion. Vitals signs and physical examination were benign without cardiac murmurs, peripheral edema, or cyanosis. Chest CT revealed absent right PA (pulmonary artery) with contrast reflux. A subsequent angiogram revealed blood flow from the right internal mammary artery to the right upper PA and from the celiac and inferior diaphragmatic artery to the right lower PA. Left and right cardiac catheterization showed normal pressures in the right atrium, right ventricle, and PA. Mean PA pressure was 14 mmHg, and wedge pressure was 6 mmHg. Left ventricular end diastolic pressure was 6 mmHg, with normal coronary artery and vein anatomy. Oxygen saturations were: SVC 86%, RA 82%, RV 81%, left PA 81%, and femoral artery 96%. Treadmill stress test was inconclusive due to early termination as the patient did not achieve 85% of maximum heart rate. Occupational medicine recommended HAST (high altitude simulation testing) in concordance with FAA regulations. Before HAST was performed, the patient opted out of his dream career of becoming a pilot. DISCUSSION: PA-IVS is a rare congenital heart disease accounting for < 1 % of total heart defects [2]. It is characterized by atresia of the RV outflow tract. The precise cause is unclear, but thought to be related to an insult during embryological development. PA-IVS can be classified into membranous and muscular forms with the former harboring a better long term prognosis. The muscular form is associated with abnormal connections between the RV and coronary artery. In our patient, left heart catheterization did not reveal abnormal coronary anatomy to suggest muscular PA-IVS.HAST is utilized in patients with cardiopulmonary defects to predict physiological response to hypobaric conditions at higher altitudes. The patient is asked to breathe a mixture of gases with an oxygen saturation of 15.1%, and simulates cabin pressures up to 2400 meters. The test is considered abnormal if there is evidence of hypoxemia (pAO2 < 50 mmHg), symptoms, and/or arrhythmias. We believe that our patient would have had an appropriate response to HAST due to a favorable form of PA-IVS and normal cardiopulmonary pressures and saturations. CONCLUSIONS:In adolescents with congenital cardiopulmonary defects a thorough workup including HAST is recommended prior to allowing exposure to a suboptimal environment.

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