Concluded Case

28 yrs male known kyphoscoliotic , presented with chronic cough dyspnea since last 1yr. increased since last 10days with pedal edema. spo2 50%on room air and with o2 support reaches to 83%. HR- around 125/min. echo sugg of sev PAH with RA RV dilatation with noncollapsing IVC. CTPA- no pulm thromboembolism. WBC 12300 on antibiotics bronchodilators mucolytics and high dose of diuretics. plz discuss further plan of action.

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Concluded answer
Sev PAH. Start with oral ambrisentan 5mg and tadalafil 20mg.. Along with bronchodilators,, mucolytics,, expectorant,, broad spectrum antibiotics..!!
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CT chest shows bilateral diffuse ground glass haziness with mosaic perfusion predominantly basal...massive cardiomegaly with posterolateral minimal pericardial effusion...kyphoscoliosis + the patient has cardiogenic pulmonary edema.. Since pulmonary embolism has been ruled out by CTPA, other trigger for cardiogenic edema should be sought...one should always rule out MI ..because this situation doesn't explain diastolic dysfunction and severe pah in a young lad even though lv function is ok.. Manage with diuretics...give empirical antiplatelets and clexane...plan an CAG soon...
Cardiogenic edema occurs in backward left heart failure commonly. LVF or MS..here there is destruction of lungs ie restrictive disease coursing PAH followed by RA and RV dllatatiion and thereby hepatic and IVC congestion ....Treatment supportive..
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Left lower lobe collapse Fibrocalcified lesion and parenchymal infiltration present in both lungs Cardiomegaly PAH present Leucocytosis present, suggest bacterial infection Broad spectrum antibiotics like meropenum, Mucolytics bronchodilaters LABA LAMA DIURETICS
Thank you doctor
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Bosenton table. Start with 32mg then increase to 62.5mg.can add other drugs like seldinafil and L Arginine. If nothing works go for heart lung transplant.
Thanks sir for your valuable comments. Started tab sildenafil.
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Sev PAH. Start with oral ambrisentan 5mg and tadalafil 20mg.. Along with bronchodilators,, mucolytics,, expectorant,, broad spectrum antibiotics..!!
Thank u dr rishi
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ADVISABLE.. 1. Bosenton 2. Sildenafil 3. L. ARGININE
Agree with Dr Srikant Thunga.
Thanks Dr Dinesh Gupta
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Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!
Thanks
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Taxhycardic with Restrictive lung disease with cor pulmonale Kindly consider avoiding saba laba and methyl xanthenes LAMA+ICS Diuretics O2 with according to abg
Nice case..Lung pathology hypoxia(long standing) causing Rt heart failure ....aggravated by infection...Spirometry will be informative ..
Kyphoscoliosis cause restrictive lung disease. Findings are suggestive of cor pulmonale. Diuretics And bronchodilators will help.
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