Combination of PGE1 and Pulmonary Vasodilator Therapy in Managing a Challenging Case of Severe PAH Secondary to CDH

2020 ◽  
Vol 11 (4) ◽  
pp. 525-527
Author(s):  
Othman A. Aljohani ◽  
Rohit Rao ◽  
Duncan Mackie ◽  
Shylah Haldeman ◽  
Tara Karamlou
Keyword(s):  
Right Ventricular ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Right Ventricular Dysfunction ◽  
Vasodilator Therapy ◽  
Venoarterial Extracorporeal Membrane Oxygenation ◽  
Pulmonary Vasodilator ◽  
Pulmonary Parenchyma ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.

scholarly journals International recommendations on neonatal management with congenital diaphragmatic hernia: literature review

2021 ◽  
pp. 51-60
Author(s):  
А.G. Babintseva ◽  
◽  
Yu.D. Godovanets ◽  
Yu.Yu. Khodzinska ◽  
N.O. Popeliuk ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Large Scale ◽  
Level Of Evidence ◽  
Vasodilator Therapy ◽  
Membrane Oxygenation ◽  
Pulmonary Vasodilator ◽  
Extracorporal Membrane Oxygenation ◽  
Ultrasound Diagnostics

Congenital diaphragmatic hernia (CDH) is a defect of diaphragm development which is characterized by herniation of the abdominal content into the chest resulting in pulmonary hypoplasia and pulmonary hypertension of various degrees. Mortality and sickness rates among survived children with this pathology remain high all over the world. Prenatal diagnostics of high quality will help to find neonates with severe CDH and promote mobilization of human and material resources for optimal management at the tertiary level of giving medical aid. Attention of specialists in prenatal ultrasound diagnostics should be drawn to the necessity of measuring observed/expected lung(to(head ratio and location of the liver in fetus. Optimal treatment of neonates during preoperative period is focused on mild ventilation, avoidance of oxygen toxicity, baro/volutrauma of a hypoplastic lung and cardiac support. Administration of pulmonary vasodilator therapy including inhaled nitric oxide and extracorporal membrane oxygenation is pathogenetically substantiated for patients with pulmonary hypertension, and surgical correction should be delayed till stabilization of patient's condition. Echocardiography is a critically important diagnostic method. It enables to determine severity of pulmonary hypertension and myocardial functional disorders, to find targeted systemic methods of treatment that improve hemodynamic function or decrease pulmonary vascular resistance. Considering an insufficient level of evidence of the existing international recommendations, large(scale multi(center randomized studies on investigation of the best methods to prevent, diagnose and treat neonates with CDH are essential. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia; neonate; lung-to-head ratio; pulmonary hypertension; extracorporal membrane oxygenation.

BIOMECHANICAL APPROACH TO THE CLINICAL TREATMENT OF PULMONARY ARTERIAL HYPERTENSION

2013 ◽  
Vol 13 (05) ◽  
pp. 1340005 ◽  
Author(s):  
IVAN CORAZZA ◽  
CHIARA PINARDI ◽  
LUIGI MANCO ◽  
DAVID BIANCHINI ◽  
LAURA CERCENELLI ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Strain Curve ◽  
Right Ventricular Dysfunction ◽  
Mechanical Efficiency ◽  
Arterial Elastance ◽  
Pulmonary Arterial ◽  
Ventricular Efficiency ◽  
Severe Pulmonary Arterial Hypertension

Negative clinical trend of severe pulmonary arterial hypertension (PAH) is due to right ventricular chamber dilatation and consequent dysfunction. On a biomechanical basis the clinical evolution is associated to a progressive mismatching of the artero-ventricular junction and a possible mechanical intervention may be foreseen. This paper demonstrates how the progressive right ventricular dysfunction, in terms of biochemical–mechanical efficiency, is related to the stress–strain curve of the pulmonary artery, simulated by superimposing different elastance tubes in a cardiovascular mock. Applying the experimental signals to an "ideal" normal and dilated ventricle, it was demonstrated that the very high pulmonary elastance at high pressure is the prime cause of a sharp efficiency drop, in comparison with an aortic similar condition. This evidence supports the proposal of mechanical interventions to reduce pulmonary arterial elastance to improve the artero-ventricular matching, increase the reduced ventricular efficiency and change the negative trend of the illness.

CONTEMPORARY NEONATAL INTENSIVE CARE MANAGEMENT IN CONGENITAL DIAPHRAGMATIC HERNIA: DOES THIS OBVIATE THE NEED FOR FETAL THERAPY?

2009 ◽  
Vol 20 (4) ◽  
pp. 319-339 ◽  
Author(s):  
DESMOND BOHN
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Case Reports ◽  
Rescue Therapy ◽  
Intervention Strategy ◽  
Case Series ◽  
High Volume ◽  
Vasodilator Therapy ◽  
Operative Stabilization ◽  
Pulmonary Vasodilator

The management of congenital diaphragmatic hernia (CDH) in the newborn infant has changed radically since the first successful outcomes were reported 60 years ago. Then it seemed a surgical problem with a surgical solution – do an operation, remove the intestines and solid viscera from the thoracic cavity, repair the defect and allow the lung to expand. CDH in that era was regarded as the quintessential neonatal surgical emergency. The expectation was that urgent surgery would result in improvement in lung function and oxygenation. That approach persisted up to the 1980s when it was realized that the problem was far more complex and involved both an abnormal pulmonary vascular bed as well as pulmonary hypoplasia. The use of systemically delivered pulmonary vasodilator therapy, principally tolazoline, became a focus of interest in the 1980s with small case reports and case series suggesting improved survival. In the 1990s, based on studies that showed worsening thoracic compliance and gas exchange following surgical repair, deferred surgery and pre-operative stabilization became the standard of care. At the same time extracorporeal membrane oxygenation (ECMO) was increasingly used either as part of pre-operative stabilization or as a rescue therapy after repair. Other centres chose to use high frequency oscillatory ventilation (HFOV). Despite all these innovations the survival in live born infants with CDH did not improve to more than 50% in large series published from high volume centres. However, in the past 10 years there has been an appreciable improvement in survival to the extent that many centres are now reporting survival rates of greater than 80%. Probably the biggest impact on this improvement has been the recognition of the role that ventilation induced lung injury plays in mortality and the need for ECMO rescue. This has ushered in an era of a lung protective or “gentle ventilation” strategy which has been widely adopted as a standard approach. While there have been these radical changes in postnatal management attempts have been made to improve outcome with prenatal interventions, starting with prenatal repair, which was abandoned because of preterm labour. More recently there has been increasing experience in the use of balloon occlusion of the trachea as a prenatal intervention strategy with patients being selected based on prenatal predictors of poor outcome. This approach can only be justified if those predictors can be validated and the outcomes (death or serious long term morbidity) can be shown to be better than those currently achievable, namely 80% survival in high volume CDH centres rather than the 50–60% survival frequently quoted in historical papers.

Mechanisms of right ventricular failure

ESC CardioMed ◽  
2018 ◽  
pp. 2493-2495
Author(s):  
Joanne A. Groeneveldt ◽  
Anton Vonk Noordegraaf ◽  
Frances S. de Man
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Dysfunction ◽  
Right Ventricular Failure ◽  
Ventricular Failure ◽  
Adaptive Mechanisms ◽  
Patient Will ◽  
Pulmonary Arterial ◽  
The Right

In pulmonary arterial hypertension, afterload on the right ventricle is increased. Due to several adaptive mechanisms, the right ventricle is able to cope with a three- to fivefold increase in afterload. However, when adaptive mechanisms are no longer sufficient to compensate for this increase, the patient will develop right ventricular dysfunction and failure. This chapter provides an overview of mechanisms currently considered as having important roles in right ventricular adaptation and right ventricular failure.

scholarly journals Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure

2020 ◽  
Vol 41 (8) ◽  
pp. 1651-1659
Author(s):  
Ida Jeremiasen ◽  
Karin Tran-Lundmark ◽  
Nikmah Idris ◽  
Phan-Kiet Tran ◽  
Shahin Moledina
Keyword(s):  
Arterial Pressure ◽  
Single Ventricle ◽  
Pulmonary Arterial Pressure ◽  
Vasodilator Therapy ◽  
Single Ventricle Physiology ◽  
Pulmonary Vasodilators ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Vasodilator ◽  
Pulmonary Arterial ◽  
Stage 1

AbstractIn children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.

scholarly journals 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

2020 ◽  
Vol 9 (2) ◽  
pp. 464 ◽  
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.

Sign in / Sign up

Export Citation Format

Share Document