A 45 years old female patient presented to our emergency department with complaints of shortness of breath for the past eight days, gradually progressive, exertional in nature without orthopnea and paroxysmal nocturnal dyspnea, cough with sputum production for five days, and two episodes of epistaxis in the past one day, which got relieved on pinching the nose. She also complained of dull aching, nonspecific abdominal pain in the past one month in the right upper abdominal region, and a history of long-standing swelling over the left calf region that has increased since one month. There was no history of chest pain, hemoptysis, fever, pedal edema, burning micturition, and bowel abnormality. She was not a known case of hypertension and diabetes. Her past history was suggestive of chullah exposure for 30 years, old treated pulmonary tuberculosis 20 years back, had multiple episodes of epistaxis since childhood period, not severe enough to require a blood transfusion, which got relieved on its own and repeated hospital visits due to pulmonary symptoms. Family and personal history were insignificant; however, she had recently been prescribed a salbutamol metered-dose inhaler (MDI) on an SOS basis. On presentation, her blood pressure was 124/84 millimeters of mercury, pulse rate of 105 / min in the right radial artery, respiratory rate of 17 /min, the random blood sugar of 213 mg/dL, and oxygen saturation of 92% under ambient air. On general physical examination, she was afebrile, pallor was noticed on lower palpebral conjunctiva, clubbing, hexadactyly of bilateral feet was noted, and hemorrhagic lesions were noted in bilateral hands and upper chest of the patient, Fig (1). A swelling was also noted in the left leg over the calf region, soft and pulsatile, with an audile bruit heard over auscultation.
On systemic examination, bruits were heard over the right inframammary, interscapular, and tricuspid area, bilateral coarse crepitations over the infra scapular area were noted, and mild tenderness was also noted in the right hypochondrium area, and the rest of the systemic examination was within normal limits. Patients' electrocardiograph was suggestive of right ventricular strain, right axis deviation, and 'p' pulmonale with sinus tachycardia, and a chest radiograph was suggestive of bilateral lower respiratory tract infection with query right lower and middle zone mass lesion (fig2). An arterial and venous doppler was performed, which showed an A-V malformation with no thrombus over the left calf region. The patient's routine blood parameters and RT-PCR swab (nasopharyngeal and oropharyngeal) for SARS-CoV-2 were send along with ABG (arterial blood gas), which showed type1 respiratory failure. Patients deranged blood parameters have been tabulated in the table (table1); however, liver function tests, serum creatine phosphokinase-total/MB fraction, sodium, potassium, amylase, and lipase remained within normal limits during the further course of hospital stay and her serology for HIV, hepatitis B, and C was negative. On admission, a provisional diagnosis of bilateral lower respiratory tract infection with a COVID-19 suspect with query pulmonary artery hypertension with query right lung mass lesion with query heart failure was made. Patients' blood and urine cultures and sputum for gram stain, acid-fast bacilli, fungal stain, and culture and sensitivity were collected and sent for analysis before augmenting intravenous antimicrobials. The patient's treatment began with oxygen support via venturi mask at 6 liters per minute, intravenous antibiotics as injectable ceftriaxone and tablet azithromycin, and blood transfusion of one unit of packed cell volume because of iron deficiency anemia. On the second day, her 2-D Echo and Contrast-Enhanced Computed Tomography (CECT) of chest and abdomen along with angiography (CT Angio) were performed. Transthoracic 2-D Echo showed a normal echo study with 60% ejection fraction, and CT Angio of the chest with the abdomen (Fig 3.) showed pulmonary artery hypertension with multiple pulmonary arteriovenous malformations in the right middle and bilateral lower lobes with hepatic, splenic, and uterine arteriovenous malformations and telangiectasia feature suggestive of Render-Osler-Weber Syndrome. Patients RT-PCR for SARS-CoV-2 reported positive; hence the patient was transferred to COVID-ICU with the addition of intravenous steroids as methylprednisolone and prophylactic anticoagulation with LMWH (lower molecular weight heparin), enoxaparin, at a dose of 0.5mg/kg subcutaneously daily, with regular monitoring of PT/INR (prothrombin time/ International Standardized Ratio), in the treatment. During the further course of hospital stay, the patient's respiratory discomfort increased, and her oxygen requirement increased, oxygen saturation dropped to 86% on room air, and ABG showed paO2 of 71 mmHg (83 – 108mmHg) around the fifth day; hence she was shifted to BIPAP (Bilevel Positive Airway Pressure) noninvasive mode of ventilation. The same day, blood investigations showed sepsis with acute kidney injury (table1); hence her antibiotics were upgraded to injection piperacillin-tazobactam combination in renal modified doses and linezolid. However, her blood, urine, and sputum cultures were reported as sterile. The patient stayed for 12 days in ICU, where she received one more unit of packed cell volume and injection meropenem along with prophylactic anticoagulation with enoxaparin, after which she was transferred back to ward on intermittent BIPAP support for 12 hours daily with NRB (Non rebreathing mask) at 10-12 liters per minute of oxygen support, as she reported negative for SARS-CoV-2. After stabilizing her in the ward for three more days and counseling her for further evaluation of the disease in the pulmonary department, the patient requested discharge on home-based long-term oxygen support because of persistence of type 1 respiratory failure as post-COVID-19 sequelae. Written informed consent was taken from the patient and her relatives for presenting her clinical data