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CLEAR FILTERS
Pulmonary arterial hypertension: new challenges in long term survivors
Session:
Prémio Melhor Caso Clínico
Speaker:
Joana Guimarães
Congress:
CPC 2023
Topic:
P. Other
Theme:
37. Miscellanea
Subtheme:
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Session Type:
Sessão de Prémios
FP Number:
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Authors:
Joana Guimarães; Diogo Fernandes; Graça Castro; Lino Gonçalves
Abstract
<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><u>Introduction:</u> Patients with pulmonary arterial hypertension (PAH) may develop chest pain which is often associated with right ventricular ischemia. However, in long term survivors, other causes should be considered, such as extrinsic compression of left main coronary artery (LMCA) by a pulmonary artery aneurysm (PAA).</span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><u>Case Report:</u> A 56 year-old female patient was diagnosed with anorexigen-associated PAH twenty-years ago. In 2018 she started complaining of typical angina on exertion with increasing frequency and intensity. Risk evaluation consistently showed a low risk score in the last 5 years. She was on triple combination therapy (ambrisentan 5mg od, sildenafil 50mg tid and treprostinil sc 75ng/kg/min). A complete study was undertaken including ECG, echocardiogram, CT coronary angiography (CTCA) and coronary angiography. A previously known pulmonary artery aneurism had expanded to 80 mm of diameter and was now causing compression of LMCA, without obstructive intracoronary lesions. LMCA stenosis was treated with a Megatron 4.5x16mm stent providing safer vessel patency. After presenting an initial improvement for the first 3 months, her angina worsened and was now triggered by minimal efforts. Further evaluation, with new echocardiogram and CTCA, showed patency of LMCA stent with PAA causing compression of medium left anterior descending artery and left ventricle. After Heart Team meeting discussion, the decision was to perform surgical aneurysmectomy, pulmonary artery and main branches replacement with a Dacron tube and preservation of the pulmonary valve by T. David technique. Surgery proceeded without complications. However, during postoperative period, the patient developed cardiogenic shock due to <em>de novo</em> left ventricular systolic dysfunction, requiring mechanical circulatory support with ECMO and an intra-aortic balloon pump. Few days later, the patient began to experience abundant digestive and uterine bleeding, leading to worsening of her clinical condition. Unfortunately, in sequence of these events, the patient passed away 13 days after surgery.</span></span></span></p> <p style="text-align:justify"> </p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><u>Conclusion:</u> Long term PAH survivors are posing new challenges namely large PAA as a potential cause of extrinsic coronary and cavity compression. A high degree of suspicion should be based, not only on symptoms, but also on image findings. The best therapeutic approach is still a matter of debate and hybrid or sequential approach may be necessary in some cases. </span></span></span></p>
Slides
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