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Unknown atrial septal defect was diagnosed during the course of treatment for portopulmonary hypertension: A case report

      Abstract

      Portopulmonary hypertension (POPH) is a condition defined by the development of pulmonary arterial hypertension (PAH) that is associated with liver disease or portal hypertension. Untreated POPH has a poor prognosis. POPH can be treated with PAH-targeted therapy and can sometimes resolve with liver transplantation (LT); however, poorly controlled POPH can preclude LT owing to an increased peri-operative risk. We report a case of POPH that was diagnosed as an unknown atrial septal defect during PAH-targeted therapy. As observed in our case, the same patient may have several causes of PAH. It is important to confirm an unknown shunt disease using echocardiography during PAH-targeted therapy.

      Learning objective

      It is difficult to detect unknown shunt disease in patients who are diagnosed with pulmonary arterial hypertension (PAH) owing to other causes. We report a case of portopulmonary hypertension that was diagnosed as an unknown atrial septal defect during PAH-targeted therapy. This report highlights the same patient may have several causes of PAH. It is important to confirm an unknown shunt disease using echocardiography during PAH-targeted therapy.

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      References

        • Simonneau G.
        • Montani D.
        • Celermajer D.S.
        • Denton C.P.
        • Gatzoulis M.A.
        • Krowka M.
        • Williams P.G.
        • Souza R.
        Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
        Eur Respir J. 2019; 53: 1801913
        • Savale L.
        • Guimas M.
        • Ebstein N.
        • Fertin M.
        • Jevnikar M.
        • Renard S.
        • Horeau-Langlard D.
        • Tromeur C.
        • Chabanne C.
        • Prevot G.
        • Chaouat A.
        • Moceri P.
        • Artaud-Macari É.
        • Degano B.
        • Tresorier R.
        • et al.
        Portopulmonary hypertension in the current era of pulmonary hypertension management.
        J Hepatol. 2020; 73: 130-139
        • Swanson K.L.
        • Wiesner R.H.
        • Nyberg S.L.
        • Rosen C.B.
        • Krowka M.J.
        Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups.
        Am J Transplant. 2008; 8: 2445-2453
        • Krowka M.J.
        • Plevak D.J.
        • Findlay J.Y.
        • Rosen C.B.
        • Wiesner R.H.
        • Krom R.A.
        Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation.
        Liver Transpl. 2000; 6: 443-450
        • Baumgartner H.
        • De Backer J.
        • Babu-Narayan S.V.
        • Budts W.
        • Chessa M.
        • Diller G.P.
        • Lung B.
        • Kluin J.
        • Lang I.M.
        • Meijboom F.
        • Moons P.
        • Mulder B.J.M.
        • Oechslin E.
        • Roos-Hesselink J.W.
        • Schwerzmann M.
        • et al.
        2020 ESC guidelines for the management of adult congenital heart disease.
        Eur Heart J. 2021; 42: 563-645
        • Shub C.
        • Dimopoulos I.N.
        • Seward J.B.
        • Callahan J.A.
        • Tancredi R.G.
        • Schattenberg T.T.
        • Reeder G.S.
        • Hagler D.J.
        • Tajik A.J.
        Sensitivity of two-dimensional echocardiography in the direct visualization of atrial septal defect utilizing the subcostal approach: experience with 154 patients.
        J Am Coll Cardiol. 1983; 2: 127-135
        • Iliceto S.
        • Antonelli G.
        • Sorino M.
        • Ricci A.
        Detection of atrial septal defect by right sternal border echocardiography.
        Am J Cardiol. 1984; 54: 376-378
        • Soliman O.I.
        • Geleijnse M.L.
        • Meijboom F.J.
        • Nemes A.
        • Kamp O.
        • Nihoyannopoulos P.
        • Masani N.
        • Feinstein S.B.
        • Ten Cate F.J.
        The use of contrast echocardiography for the detection of cardiac shunts.
        Eur J Echocardiogr. 2007; 8: S2-S12
        • Osawa K.
        • Miyoshi T.
        • Morimitsu Y.
        • Akagi T.
        • Oe H.
        • Nakagawa K.
        • Takaya Y.
        • Kijima Y.
        • Sato S.
        • Kanazawa S.
        • Ito H.
        Comprehensive assessment of morphology and severity of atrial septal defects in adults by CT.
        J Cardiovasc Comput Tomogr. 2015; 9: 354-361
        • Deroo R.
        • Trépo E.
        • Holvoet T.
        • De Pauw M.
        • Geerts A.
        • Verhelst X.
        • Colle I.
        • Van Vlierberghe H.
        • Fallon M.B.
        • Raevens S.
        Vasomodulators and liver transplantation for portopulmonary hypertension: evidence from a systematic review and meta-analysis.
        Hepatology. 2020; 72: 1701-1716