SPC365

Never Stop Looking - The Heart is Not Always the Answer!

Session: Casos Clínicos: Insuficiência Cardíaca e Cuidados Intensivos Cardíacos
Speaker: Daniel Inácio Cazeiro

Congress: CPC 2024
Topic: D. Heart Failure
Theme: 10. Chronic Heart Failure
Subtheme: 10.6 Chronic Heart Failure - Clinical
Session Type: Sessão de Casos Clínicos
FP Number: ---
Authors: Daniel Inácio Cazeiro; Miguel Azaredo Raposo; Ana Abrantes; Catarina Gregório; Diogo Ferreira; João Cravo; Marta Vilela; Susana Robalo Martins; Nuno Lousada; Tatiana Guimarães; Fausto J. Pinto; Rui Plácido

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:11.0pt"><span style="color:#222222">Background: </span></span></strong><span style="font-size:11.0pt"><span style="color:#222222">Pulmonary hypertension (PH) associated with congenital heart disease (CHD) falls under group I classification of PH. While commonly linked to defects such as atrial/ventricular septal defects, patent ductus arteriosus, and anomalous pulmonary venous return, there are rarer forms of CHD warranting investigation when clinical suspicion is high.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:11.0pt"><span style="color:#222222">Case summary</span></span></strong><span style="font-size:11.0pt"><span style="color:#222222">: A 63-year-old female, a former smoker with a history of systemic arterial hypertension and non-significant coronary artery disease, presented with worsening fatigue and dyspnea. High echocardiographic probability of PH prompted referral to a PH expert centre. She was a former waitress and had no relevant occupational exposure. Family history was unremarkable. A comprehensive work-up ensued, ruling out autoimmune disease, HIV and hepatitis, and revealing no significant findings in ventilation/perfusion scan, lung computed tomography angiography (angioCT), pulmonary function tests, and abdominal ultrasound.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:11.0pt"><span style="color:#222222">A more comprehensive transthoracic echocardiogram was done, depicting mild left ventricular D-shape with preserved ejection fraction, 4-chamber dilation and severe tricuspid regurgitation (peak gradient of 54 mmHg). </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:11.0pt"><span style="color:#222222">Right heart catheterization confirmed a diagnosis of PH, with a pre-capillary phenotype [mean pulmonary artery pressure (mPAP) of 34 mmHg and pulmonary vascular resistance (PVR) of 3 Wood units (WU)], as well as high cardiac output (CO) (12 L/min). The absence of significant oxygen saturation step-up between the superior vena cava and the pulmonary artery excluded intracardiac shunt.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:11.0pt"><span style="color:#222222">These findings, along with the absence of congenital anomalies in chest angioCT, raised suspicion for a significant extra-thoracic left-to-right shunt. Therefore, a full body angioCT scan was ordered, revealing an arteriovenous fistula (AVF) between the posterior wall of the right common iliac artery and the confluence of both common iliac veins. </span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:11.0pt"><span style="color:#222222">The AVF was successfully corrected via an endovascular procedure, resulting in an immediate resolution of symptoms.</span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:11.0pt"><span style="color:#222222">Discussion:</span></span></strong><span style="font-size:11.0pt"><span style="color:#222222"> This rare case underscores an unusual cause of PH associated with CHD, where an extrathoracic AVF induced a high CO state, subsequently elevating right ventricular preload, mPAP, and PVR, ultimately manifesting as symptoms of high-output ad congestive heart failure. Timely diagnosis is crucial, enabling corrective intervention prior to the emergence of significant structural heart disease and associated morbimortality. The case emphasizes the significance of a thorough clinical investigation in patients with PH, urging medical practitioners to consider less common causes for a comprehensive understanding and effective management of this complex condition.</span></span></span></span></p>