Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry

Duffels, Marielle G.; Engelfriet, Peter; Berger, Rolf M. F.; Loon, Rosa L. E. van; Hoendermis, Elke S.; Vriend, Joris W.J.; Velde, Enno T. van der; Bresser, Paul; Mulder, Barbara J.M.

doi:10.1016/j.ijcard.2006.09.017

Cited by 293 publications

(216 citation statements)

References 22 publications

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“…Among adult patients with CHD, the reported prevalence of PAH is 4-28% and of Eisenmenger syndrome (ES) is 1-6%. 4,5) These reported prevalences indicate that there may be 16,000-112,000 adult patients with PAH associated with CHD in Japan, and 4,000-24,000 adult patients with ES. It is important to establish guidelines for the management of these patients, which requires establishment of a register of adult patients with PAH associated with CHD.…”

Section: Prevalence Of Pah Associated With Chdmentioning

confidence: 97%

Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

Nakanishi

2015

Int. Heart J.

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SummaryThe management of patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy with selective pulmonary vasodilators. The number of adult Japanese patients with PAH associated with CHD is increasing. It is important to develop evidence-based guidelines for the management of these patients, and to achieve this, a register of adult Japanese patients with PAH associated with CHD should be established. At the World Symposium in Nice, France, in 2013, the consensus was reached that patients with a pulmonary resistance of < 4 Wood Units (WU)·m 2 have operable disease, and patients with a pulmonary resistance of > 8 WU·m 2 have inoperable disease. However, these criteria are conservative. Some patients with a pulmonary resistance of > 8 WU·m 2 and a good response to a pulmonary vasodilator test have operable disease and a favorable clinical course long after repair of CHD. The criteria determining operability in patients with PAH associated with CHD in the era of pulmonary vasodilators should be established using data obtained from patient registers and/or multicenter studies. The optimal management of Eisenmenger syndrome should also be established using data obtained from patient registers. Prospective studies should be conducted to determine the life expectancy of patients with Eisenmenger syndrome in the era of targeted therapy. A relatively mild increase in pulmonary resistance may result in failure of a Fontan circulation. The effects of pulmonary vasodilators on the long-term prognosis of patients who have undergone the Fontan operation are still unclear. (Int Heart J 2015; 56: S1-S3) Key words: Target therapy, Pulmonary vasodilator, Eisenmenger syndrome, Fontan operation, Pulmonary resistance T he management of patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy using selective pulmonary vasodilators. Many studies have reported on the efficacy of targeted therapy for PAH associated with CHD.1) This special issue reviews recent clinical studies of patients with PAH associated with CHD, and various aspects of the pathophysiological mechanisms underlying this condition. Here, I briefly summarize my perspectives regarding PAH associated with CHD as an introduction to this special issue. Definition and ClassificationIn 2013, a new classification system for PAH associated with CHD was announced, based on the expert consensus reached at the World Symposium in Nice, France, in 2013 (Table I).2) This classification system is useful when discussing patients with PAH associated with CHD.PAH is defined as a mean pulmonary arterial (PA) pressure of ≥ 25 mmHg in association with a PA wedge pressure or left atrial pressure of ≤ 15 mmHg.3) This definition applies regardless of the age of the patient, and is accepted in pediatric and adult medicine. As the systemic arterial pressure is lower in young children, especially in infants...

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Section: Prevalence Of Pah Associated With Chdmentioning

confidence: 97%

Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

Nakanishi

2015

Int. Heart J.

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“…There are four types of VSDs: subarterial (or supracristal), perimembranous (or infracristal), inlet (or canal type), and muscular [14 • ] A solitary VSD is a common congenital heart defect; however, VSDs also occur in 50 % of complex CHD. A VSD can be seen in association with chromosomal abnormalities such as trisomy 13, 18, and 21 [15]. The inter-ventricular communication seen in unrepaired VSDs causes a left to right shunt at the ventricular level during systole.…”

Section: Ventricular Septal Defectmentioning

confidence: 99%

Perioperative Care of Adults with Congenital Heart Disease for Non-cardiac Surgery

Howard‐Quijano

Smith

Schwarzenberger

2013

Curr Anesthesiol Rep

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In 2010 it was estimated that 1.2 million young adults in the United States have some form of congenital heart disease (CHD). Data from large adult congenital heart disease (ACHD) centers show that as many as 50 % of these patients are over 40 years of age. As this population ages, physicians are likely to encounter such patients in their practice. Risk stratification and an interdisciplinary approach are paramount in achieving safe outcomes. The assessment of the patient must consider the variable expression of CHD. As most lesions are ''fixed but not cured'' the altered physiology of the heart and the physiological challenges of surgical interventions must be considered. The 2008 AHA/ACC guidelines for the Management of Adults with CHD establish for the first time a streamlined approach to the care of these patients. This review will apply these guidelines to the ACHD patient undergoing non-cardiac surgery.

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“…22,33 Some studies estimate 4% to 10% of all patients with CHD develop Eisenmenger syndrome; however, this increases to as high as 30% in patients with unrepaired congenital defects. [33][34][35] Worldwide, it is estimated that 3.2 million children are at risk for the development of PAH-CHD. 24 Most of them do not develop Eisenmenger syndrome, particularly if cardiac repair occurs within the first 2 years of life.…”

Section: Epidemiologymentioning

confidence: 99%