Role and management of extracorporeal life support after surgery of chronic thromboembolic pulmonary hypertension.

BACKGROUND: Pulmonary endarterectomy (PEA) is a surgical intervention reserved for patients with chronic thromboembolic pulmonary hypertension (CTEPH). In some cases, temporary circulatory support [extracorporeal life support (ECLS)] is required after PEA. Rates of ECLS requirement varies between centers. Reasons for institution of ECLS include respiratory failure, cardiac failure (or both respiratory and cardiac failure), bleeding, and reperfusion edema. This article reviews the experience of ECLS after PEA from the current literature, as well as our own institution's experience as a CTEPH multidisciplinary center. METHODS: A literature review was conducted along with a retrospective chart review from 15 years of our PEA program. RESULTS: The literature demonstrates many different approaches are used for mechanically supporting patients who develop complications after PEA. Variations in approach stem from differing indications such as, respiratory failure rather than hemodynamic compromise (or vice versa), time of implantation (immediately in operating room or delayed after surgery) and many other causes. In our center, 12.3% (19/154) of patients need ECLS with extracorporeal membrane oxygenator (ECMO) after PEA procedure. Implantation was mainly in the operating room before or immediately after weaning from cardiopulmonary bypass and mostly peripheral cannulation was used. ECMO lasted an average of 11±8 days. And 52.6% (10 of 19 patients) of patients were weaned from ECLS and of this, 70% (7 of 10 patients) were discharged. CONCLUSIONS: In some cases of PEA, ECLS is needed post-operatively. Expert teams should consider this possibility pre-operatively based on predisposing characteristics. The need for ECMO shouldn't be "di per se" a contraindication to surgery but might be considered in the surgical risk estimation. The ideal setup is not fixed and depends on the center's practices as well as indication. Even though complications do occur with ECMO, in general, results are good, being a bridge to further recovery of pulmonary hypertension (PH) or also to transplantation.

Thoracic manifestation of Eisenmenger's syndrome in adult patients: a MDCT review.

Eisenmenger's syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD). It is caused by simple or complex CHD with a large systemic-to-pulmonary shunt. When the pulmonary pressure exceeds the systemic pressure, the shunt reverses and central cyanosis appears. ES is a progressive and fatal condition, and it is accompanied by an increased risk of a range of potentially life-threatening complications. Patients with ES are both at risk for bleeding, due to damaged capillaries and high pressure, and for in situ pulmonary thrombosis, related to hyper-viscosity and slow blood flow in dilated pulmonary arteries. Moreover, the enlarged main pulmonary arteries and cardiac chambers may determine displacement or extrinsic compression on airways, pulmonary veins, coronary arteries, and other mediastinal structures. The clinical effects may be diverse, such as respiratory difficulties, localized pulmonary edema, cardiac dysfunction, or sudden death. Multidetector computed tomography (MDCT) allows to accurately assess in a single examination, pulmonary parenchyma and vessels, coronary artery origin, and heart chambers. The aim of this review was to illustrate the thoracic MDCT angiography findings and complications of adult patients with PAH-CHD and in particular of those with ES.

Pulmonary veno-occlusive disease: the role of CT.

OBJECTIVE: The authors sought to evaluate the diagnostic accuracy of high-resolution computed tomography (HRCT) in the detection of pulmonary veno-occlusive disease (PVOD) in patients with pre-capillary pulmonary arterial hypertension (PAH) of unknown aetiology, and to identify the role of CT in diagnosis and therapy. MATERIALS AND METHODS: The CT scans of 96 patients were retrospectively reviewed and assessed for specific HRCT findings: ground-glass opacities, septal lines and mediastinal lymph nodal enlargement (short diameter ≥1 cm). According to the HRCT findings, patients were divided into PVOD-suspicious and not PVOD-suspicious. Subsequently, a clinical-instrumental evaluation was performed, and the response to therapy and histopathological reports were evaluated. RESULTS: Radiological evaluation based on HRCT findings revealed 29 patients as PVOD-suspicious and 67 as not PVOD-suspicious. The final diagnosis was PVOD in 22 patients and idiopathic PAH in 74 patients. The CT scan showed 95.5 % sensitivity, 89% specificity, 72.5% positive predictive value, and 98.5% negative predictive value, with a diagnostic accuracy of 90.5% in identifying patients with PVOD. CONCLUSIONS: Chest CT can be considered a screening test in the assessment of patients with PAH of unknown aetiology, and the radiologist can help the clinician to identify patients with CT findings that make PVOD highly probable.