Advanced therapy for pulmonary arterial hypertension due to congenital heart disease: a clinical perspective in a new therapeutic era

Schuuring, Mark J.; Boekholdt, S. Matthijs; Windhausen, A.; Bouma, Berto J.; Groenink, Maarten; Keijzers, Mitran; Winter, R.J. de; Koolbergen, David R.; Blom, Nico A.; Mulder, Barbara J.M.

doi:10.1007/s12471-011-0218-x

Cited by 9 publications

(4 citation statements)

References 14 publications

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“…Patients with CHD-PAH often are subject to an increased pulmonary vascular resistance for a long time. As a consequence, RV overload causes RV hypertrophy and dilatation [26]. This leads to RV dysfunction and congestion [14,27,28].…”

Section: Discussionmentioning

confidence: 99%

New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study

Schuuring

Riel

Vis

et al. 2015

International Journal of Cardiology

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Section: Discussionmentioning

confidence: 99%

New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study

Schuuring

Riel

Vis

et al. 2015

International Journal of Cardiology

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“…While it is possible that patients with other forms of CHD (e.g., ASD) who are not surgical candidates because of severe PAH may derive benefits from perioperative bosentan therapy, its role in this setting is still a matter of debate. 80–82 Further study is needed to determine whether pretreatment with bosentan can influence either morbidity or mortality in patients at high risk for PH crisis.…”

Section: Endothelin Receptor Antagonists (Eras)mentioning

confidence: 99%

“…In a case report that included 8 children with univentricular physiology who were not candidates for right‐sided heart bypass because of elevated PVR, Hirono et al 79 demonstrated that use of bosentan significantly lowered PVR and allowed the successful performance of the Fontan procedure. While it is possible that patients with other forms of CHD (e.g., ASD) who are not surgical candidates because of severe PAH may derive benefits from perioperative bosentan therapy, its role in this setting is still a matter of debate 80–82 . Further study is needed to determine whether pretreatment with bosentan can influence either morbidity or mortality in patients at high risk for PH crisis.…”

Section: Endothelin Receptor Antagonists (Eras)mentioning

confidence: 99%

Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Brunner¹,

Perez²,

Richter³

et al. 2014

Pulm. circ.

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Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.

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“…The aim of intervention in patients with pulmonary hypertension associated with congenital heart disease (PH-CHD) is to optimise pulmonary flow and prevent RV failure. Targeted therapy for pulmonary hypertension consists of prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors and can improve functional status in PH-CHD patients [8][9][10]. Paradoxically, it is suggested that targeted therapy and the consequential reduction in PVR can cause an increase in pulmonary shunt flow and may result in progression of pulmonary endothelial damage [5,11].…”

Section: Introductionmentioning

confidence: 99%

Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease

et al. 2016

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Background Adults with pulmonary hypertension associated with congenital heart disease (PH-CHD) often have residual shunts. Invasive interventions aim to optimise pulmonary flow and prevent right ventricular failure. However, eligibility for procedures strongly depends on the adaptation potential of the pulmonary vasculature and right ventricle to resultant circulatory changes. Current guidelines are not sufficiently applicable to individual patients, who exhibit great diversity and complexity in cardiac anomalies. Methods and ResultsWe present four complex adult PH-CHD patients with impaired pulmonary flow, including detailed graphics of the cardiopulmonary circulation. All these patients had an ambiguous indication for shunt intervention. Our local multidisciplinary Grown-Ups with Congenital Heart Disease team reached consensus regarding a patient-tailored invasive treatment strategy, adjacent to relevant guidelines. Interventions improved pulmonary haemodynamics and short-term clinical functioning in all cases. Conclusions Individual evaluation of disease characteristics is mandatory for tailored interventional treatment in PH-CHD patients, adjacent to relevant guidelines. Both strict

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Advanced therapy for pulmonary arterial hypertension due to congenital heart disease: a clinical perspective in a new therapeutic era

Cited by 9 publications

References 14 publications

New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study

New predictors of mortality in adults with congenital heart disease and pulmonary hypertension: Midterm outcome of a prospective study

Perioperative Pharmacological Management of Pulmonary Hypertensive Crisis during Congenital Heart Surgery

Tailored circulatory intervention in adults with pulmonary hypertension due to congenital heart disease

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