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Ostium secundum atrial septal defect closure in apparently irreversible pulmonary hypertension
Session:
Sessão Speaker’s Corner - Casos clínicos desafiantes… em Cardiologia – 2
Speaker:
Mariana Martinho
Congress:
CPC 2022
Topic:
P. Other
Theme:
37. Miscellanea
Subtheme:
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Session Type:
Sessão de Casos Clínicos
FP Number:
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Authors:
Mariana Martinho; Alexandra Briosa; Filipa Ferreira; Rita Calé; João Grade Santos; Bárbara Ferreira; Diogo Santos Cunha; Nazar Ilchyshyn; João Luz; Sofia Alegria; Maria José Loureiro; Débora Repolho; Hélder Pereira
Abstract
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><span style="font-size:11.0pt">Patients with an atrial septal defect (ASD) and mild pulmonary hypertension (PH) are ideal candidates for closure, preventing disease progression. Clinical data is more controversial in cases of moderate-to-severe PH and determining PH reversibility is crucial for treatment decision. Closing ASD in patients with irreversible PH can be clinically catastrophic and is not recommended. On the other hand, not closing in patients with reversible PH deprives them of clinical improvement. Whether treatment with pulmonary artery vasodilators in this subgroup of patients is a valuable asset to promote future closure is not well understood.</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><span style="font-size:11.0pt">We present a case of 37-year-old woman with no previous medical history, who was referred to our PH-unit due to progressive worsening fatigue (functional class WHO II) and high probability of PH on a transthoracic echocardiogram – right ventricle and pulmonary trunk dilation and pulmonary artery systolic pressure of 85mmHg. Transesophageal echocardiogram revealed the presence of an ostium secundum ASD (1.86x1.34 cm) with bidirectional flux. Blood analysis and pulmonary functional test were normal. Cardiopulmonary exercise test (CPET) demonstrated decreased functional capacity (Peak VO2 17,1; 50% of predicted; desaturation from 99 to 94%). Pulmonary scintigraphy (PS) showed multiple perfusion defects, that resolved 3 months after oral anticoagulation. A right heart catheterization (RHC) confirmed the presence of precapillary severe PH.<strong> </strong>Vasoreactivity test with epoprostenol was negative, supporting irreversibility. Based in these results, ASD was not considered for closure and medical therapy with sildenafil and bosentan was started. After one year of treatment, RHC showed a significant improvement in PH, but with an increase in Qp:Qs. She was referred for surgical closure (borders not suitable for percutaneous closure) with a 4.5mm-fenestrated patch, with success. Six months after surgery, the patient was on WHO class I, improved peak VO2 24,1 mL/kg/min (81% of predicted) and RHC showed only mild PH.</span></span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><span style="font-size:11.0pt">Our patient presented several criteria considered to be a contraindication for ASD closure: a spontaneous bidirectional shunt with slight desaturation in CPET and severe PH on RHC with a negative vasoreactivity test. Closing ASD in patients with severe PH is increasingly becoming a successful reality. Our case emphasizes the importance of individual assessment even when initial evaluation is unfavorable to closure in accordance with the guidelines. Further studies are necessary to evaluate the long-term impact of these interventions.</span></span></span></p>
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