ABSTRACT
Background. Massive subchorionic thrombohematoma (MST), also known as Breus' mole, is a rare and poorly understood entity defined as a substantial collection of clotted blood in the intervillous space, immediately beneath the chorionic plate, measuring >1 cm in thickness with >50% involvement of the fetal surface of the placenta. The presumed pathophysiology is an aberrant collection of maternal blood, although this data is limited. MST has previously been associated with maternal thrombophilia and following thrombolytic therapy. Method(s): Four cases of MST diagnosed by pathological examination at our institution from 1/1/2021-7/1/2022 were identified using our laboratory information system. Maternal medical history, prenatal imaging, antenatal complications, gestation at delivery, and pregnancy outcome were extracted from the medical record. Result(s): We report a novel association of MST and maternal cardiac dysfunction, illustrated by four cases at a highrisk obstetric reference center covering a large portion of the southeastern United States. Two of these mothers had surgically repaired complex congenital heart disease, one had heart failure secondary to SARS-CoV-2 myocarditis, and one had longstanding high output heart failure due to severe sickle cell disease with resulting severe anemia. All of these pregnancies were complicated by intrauterine growth restriction (IUGR). Grossly, all four placentas had extensive fetal surface involvement by thrombohematoma (90-100%), but with variable degrees of placental volume replacement (range: 10-80%). Two of the four mothers had documented enoxaparin administration during pregnancy;however, there were no common medications given to all four mothers None had recognized thrombophilic disorders. Only one of four lesions was definitively identified on prenatal ultrasound. Of these four cases, three were live births, though only one infant survived past fifteen days of life. This surviving child had the least affected placenta grossly, was delivered at term, and was born to the only mother who did not require admission to the intensive care unit. Conclusion(s): We posit abnormal maternal hemodynamics are the final common pathway in the development of MST. Previous studies have shown blood within the thrombohematoma to be of maternal origin;furthermore, there is correlation within our case series between the largest lesions and the mothers with the most hemodynamic instability. IUGR is likely secondary to decreased placental reserve capacity from these space-occupying lesions. High fetal/neonatal morbidity and mortality rates underscore the need to further characterize this pathophysiologic process. That only one of four cases was detected on prenatal ultrasound illustrates the importance of both ante- and postnatal clinical and pathologic recognition.
ABSTRACT
CASE: A 51-year-old man without significant past medical history presented to our hospital with dyspnea on exertion. SARS-CoV-2 was detected on routine occupational screening 2 months prior to admission. He subsequently reported a 100lb weight loss, during which time he experienced dysgeusia and ate primarily cereal, sandwiches, and potatoes and consumed nearly no fruits or vegetables. Three weeks prior to admission he developed postprandial nausea and vomiting and anorexia. A week later he developed progressive epigastric pain, lower extremity edema, and dyspnea while walking around the college campus where he worked as a security guard, and sought medical attention. He did not have fever, chills, night sweats, cough, orthopnea, paroxysmal nocturnal dyspnea, rash, or diarrhea. He had not seen a doctor in 20 years and took no medications. He did not drink alcohol, smoke cigarettes, or use illicit substances. Vital signs were T 36.6°F HR 104 BP 149/111 RR 20 and SpO2 97%. Physical examination revealed a cachectic man with bitemporal wasting, sunken orbits, poor dentition, and severe periodontal disease. JVP was 14cm of H2O at 45°. An S3 was present. The abdomen was tender to palpation in the mid epigastrium. The extremities were cool with 3+ pitting edema. Pancreatitis was diagnosed after discovery of markedly elevated lipase levels and peripancreatic fat stranding on abdominal CT. TTE showed biventricular systolic dysfunction with LVEF 15%. He developed cardiogenic shock complicated by oliguric renal failure, congestive hepatopathy and obtundation, requiring ICU transfer for diuresis and inotropic support. Further workup revealed deficiencies of thiamine, zinc, and vitamins A, C, and D. A regadenoson myocardial perfusion PET/CT showed no flow-limiting coronary artery disease, and workup for inflammatory, infectious, and toxic-metabolic causes of heart failure was unrevealing. While COVID myocarditis and multisystem inflammatory syndrome in adults (MIS-A) were considered, ultimately, a diagnosis of wet beriberi was made. After 5 months of aggressive nutritional supplementation via percutaneous gastrostomy tube and initiation of guideline-directed medical therapy, LVEF improved to 53% and weight increased by 35lbs. IMPACT/DISCUSSION: Wet beriberi is a potentially underrecognized cause of dilated cardiomyopathy in resource-rich areas. Within 3 months, thiamine deficiency can cause high-output heart failure due to impaired myocardial energy metabolism and dysautonomia. Risk factors include alcohol use disorder, prolonged vomiting, and history of bariatric surgery. CONCLUSION: The laboratory evaluation of non-ischemic dilated cardiomyopathy should include measurement of serum thiamine, carnitine, and selenium levels in select patients, alongside iron studies, ANA, screening for HIV, Chagas disease, and viral myocarditis, and genetic testing in patients with a suggestive family history. Empiric thiamine repletion should be considered in all critically ill patients with evidence of malnutrition.