Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

We report long-term outcome after one-stage, surgical repair, in a two months-old girl with persistent truncus arteriosus type I, II. The operation was carried out with the remodeling of the right ventricle, using a swine bicuspid pulmonary prosthesis. Twenty-six years later, the patient is in excellent clinical condition, CF I (NYHA), with normal peripheral oxygen saturation. Recent invasive and not invasive imaging show: absence of intracardiac shunt and growing of the right ventricle outlet tract and discrete double lesion of the pulmonary valve. The pulmonary flow directed uniformly for both lungs. In selected cases, the long-term prognosis of patients with persistent truncus arteriosus, undergoing early surgical repair, avoiding the use of valved conduit, makes for an excellent evolution, without new interventions. Endovascular procedures, now well standardized, for the implantation of a pulmonary valve stent, through a catheter, will allow an effective approach, in the presence of late obstructions, in patients who have undergone right ventricular remodeling, without the use of valved conduits.

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Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of fallot

The Annals of Thoracic Surgery ◽

10.1016/s0003-4975(02)03586-5 ◽

2002 ◽

Vol 73 (6) ◽

pp. 1794-1800 ◽

Cited By ~ 84

Author(s):

Frank T.H. de Ruijter ◽

Ineke Weenink ◽

Francois J. Hitchcock ◽

Erik J. Meijboom ◽

Ger B.W.E. Bennink

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Valve Replacement ◽

Ventricular Dysfunction ◽

Pulmonary Valve ◽

Right Ventricular Dysfunction ◽

Pulmonary Valve Replacement

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The Clinical Impact of Flow Titration on Epoprostenol Delivery via High Flow Nasal Cannula for ICU Patients with Pulmonary Hypertension or Right Ventricular Dysfunction: A Retrospective Cohort Comparison Study

Journal of Clinical Medicine ◽

10.3390/jcm9020464 ◽

2020 ◽

Vol 9 (2) ◽

pp. 464 ◽

Cited By ~ 1

Author(s):

Jie Li ◽

Payal K. Gurnani ◽

Keith M. Roberts ◽

James B. Fink ◽

David Vines

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Gas Flow ◽

Ventricular Dysfunction ◽

Right Ventricular Dysfunction ◽

Constant Flow ◽

High Flow Nasal Cannula ◽

Refractory Hypoxemia ◽

Pulmonary Arterial ◽

Flow Titration

(1) Background: inhaled epoprostenol (iEPO) delivered via high-flow nasal cannula (HFNC) has been reported to be effective for pulmonary hypertension and right ventricular dysfunction. In vitro studies have identified HFNC gas flow as a key factor in trans-nasal aerosol delivery efficiency; however, little evidence is available on the clinical impact of flow titration on trans-nasal aerosol delivery. At our institution, iEPO via HFNC was initiated in 2015 and the concept of flow titration during iEPO via HFNC has been gradually accepted and carried out by clinicians in the recent years. (2) Methods: a retrospective review of the electronic medical records for all adult patients who received iEPO via HFNC in a tertiary teaching hospital. Pre- and post- iEPO responses were reported for patients whose HFNC flow was titrated or maintained constant during iEPO delivery. Positive response to iEPO was defined as the reduction of mean pulmonary arterial pressure (mPAP) > 10% for pulmonary hypertension patients or the improvement of oxygenation [pulse oximetry (SpO2)/fraction of inhaled oxygen (FIO2)] > 20%. The number of responders to iEPO was compared between groups with titrated vs constant flow. (3) Results: 51 patients who used iEPO to treat pulmonary hypertension and/or right ventricular dysfunction were reviewed. Following iEPO administration via HFNC, mPAP decreased (43.6 ± 11.7 vs. 36.3 ± 9.7 mmHg, p < 0.001). Among the 51 patients, 24 had concomitant refractory hypoxemia, their oxygenation (SpO2/FIO2) improved after iEPO delivery (127.8 ± 45.7 vs. 157.6 ± 62.2, p < 0.001). During iEPO initiation, gas flow was titrated in 25 patients and the remaining 26 patients used constant flow. The percentage of patients in the flow titration group who met the criteria for a positive response was higher compared to the group with constant flow (85.7% vs. 50%, p = 0.035). Pre- vs post-iEPO responses were significant in the flow titration group included improvement in cardiac output (p = 0.050), cardiac index (p = 0.021) and FIO2 reduction (p = 0.016). These improvements in hemodynamics and FIO2 were not observed in the constant flow group. (4) Conclusion: in patients with pulmonary hypertension and/or right ventricular dysfunction, trans-nasal iEPO decreased pulmonary arterial pressure. It also improved oxygenation in patients with combined refractory hypoxemia. These improvements were more evident in patients whose gas flow was titrated during iEPO initiation than those patients using constant flow.

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