A male patient, aged 45 years old, farmer, non-smoker. Complaining of dyspnaea on exertion for 3 years, cough with slight expectoration, and precordial pain. Splenectomy was done 4 years ago.
On examination, HR was 90 b/m, regular, BP 110/70, no cyanosis or clubbing of fingers. There was a pan systolic murmur over the tricuspid area and the apex, with early diastolic murmur over the pulmonary area. The chest was clear.
Attached the echocadiogram, ECG, and chest X-ray.
Interesting case...Some clarity would make for some clarification regarding as to the cause of splenectomy that has been performed (trauma or secondary hypersplenism?)
1. ECHO: severe pulmonary hypertension and right heart dilatation with severe TR, dilatation of the pulmonary trunk, flows discharge of are not visible (no atrial septal defect, no ventricular septal defect, open arterial duct is not also). TR max gradient 98,9 mm Hg. It is may be a primary pulmonary hypertension or pulmonary venous hypertension due to extrinsic compression of central pulmonary veins (fibrosing mediastinitis, adenopathy and/or tumors)? Сould it be obstruction of distal pulmonary arteries (pulmonary embolism by thrombus, tumor, ova and/or parasites, foreign material) or RV stenosis of the outflow tract?
2. ECG: there are right axis deviation and right ventricular rotation forward, RBBB. Repolarisation abnormality due to right ventricular hypertrophy or dilatation - ST depression and T wave inversion in the leads corresponding to the right ventricle, i.e right ventricular strain pattern involving the precordial V1-3 leads (T-wave inversions are seen in V1 and ST-T depression in V2-3 leads; the same findings are observed in patient with acute right ventricular dilatation due to massive pulmonary embolism) observed.
3. Chest X-Ray: a mysterious shadow in the central part of the right lung (foreign body, tumor, or parasitic cyst)
Сonclusion: 1) foreign body (less likely here for adenopathy and/or tumors) leading to extrinsic compression of central pulmonary veins inducing pulmonary hypertension. Perhaps there has been a trauma with a penetrating wound four years ago, which led to the rupture of the spleen and subsequent splenectomy. 2) It can not be excluded in this case, hemato-oncology, or sarcoidosis, possibly malaria or parasite polycystic
Thank you very much Dr. Vaikhanskaya for your useful contribution. Splenectomy was done to our patient because of hypersplenism secondary to Schistosoma mansoni infestation.
Then maybe it's cor pulmonale as the chronic form of the schistosomiasis associated with formation of granulomas around trapped Schistosoma`s eggs disseminated into the lungs and pulmonary arteries (pulmonary embolism by Schistosome eggs). How should be to help the patient in this situation?
Thank you very much Dr. Vaikhanskay, I totally agree with your diagnosis, as well as my colleagues in our center.
Since we began to treat Schistosoma patients with Praziquantel in the early 1980s, its incidence markedly decreased. The "Bilhrazial cor-pulmonale" is a very rare disease even in Egypt, the land of Bilharziasis. We searched in the literature and unfortunately we did not found any trials on treatment of such cases. We found only few expert opinions stated that the anti-Bilharzial treatment is usually ineffective, and treatment is same as primary pulmonary hypertension with a very poor outcome.
Echocardiography suggests severe pulmonary hypertension with significant right chamber enlargement. Apparently nothing is really abnormal on the left side (except possibly for a slight left atrial enlargement, which is nonetheless less than right atrial enlargement and with interatrial septal bowing towards the left atrium, suggesting that right atrial pressure is higher than left atrial pressure).
All this may suggest pre-capillary rather than post-capillary PH.
The patient has risk factors for pulmonary arterial hypertension (associated with schistosomiasis) as well as for chronic thromboembolic PH (splenectomy).
Cough and chest pain in this context suggest an advanced form of disease (in particular, chest pain with such a huge dilatation of the main trunk of the pulmonary artery may suggest extrinsic compression on the left main coronary artery).
He should undergo a complete workup for PH in order to provide him an adequate treatment.
Dr Mohammad, I think that this interesting case deserves to be published. You can submit a manuscript to the Journal of Clinical and Medical Case studies.
You may submit your paper by e-mail at [email protected]. As this is inaugural issue, your submission will be published free of cost. Submission of manuscript ends by: 25th November, 2016
Severe pulmonary hypertension on top of bilharizial core pulmonale
(Huge dumble shaped pulmonary shadow in CXR)
ECHO: Right Ventricular Pressure OverLoad with ↑SED LV eccentricity Index.
Conc: R\O Pulmonary EMBOLISM
EKG: Bifuscular Block, ST Δes V2\3 of Posterior Wall INFARCTION Pattern[DEMAND INFARCTION 2° Mssive Pulmonary EMBOLISM].
Chest XRAY: -Fleishner sign[ enlarged pulmonary artery ]
-Westermark sign [Right Sided Shadow(opacity)
Δx: Pulmonary EMBOLISM
Rx: Thrombolyse
CHEST CT Angiography results????
D-DIMERS ????