Lung Perfusion Scintigraphy in Eisenmenger Syndrome Due to Patent Ductus Arteriosus.

Eisenmenger syndrome refers to the elevation of pulmonary arterial pressure to the systemic level caused by an increased pulmonary vascular resistance with right-to-left shunt through an intracardiac or aortopulmonary communication. A 36-year-old woman with Eisenmenger syndrome due to patent ductus arteriosus underwent Tc-MAA lung perfusion scintigraphy to evaluate right-to-left shunt. Whole-body imaging visualized extrapulmonary activity in both kidneys, spleen, and intestinal tract, confirming the presence of right-to-left shunt. Accumulation in the brain was visible but much weaker compared with that in the body trunk and was limited to the left cerebral hemisphere, which reflected the location of the shunt pathway.

Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.

Hipertensión pulmonar asociada a cardiopatías congénitas y síndrome de Eisenmenger / Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome

La hipertensión arterial pulmonar es una complicación frecuente de las cardiopatías congénitas (CC). Es reconocido que las CC son las malformaciones más frecuentes al nacimiento con una prevalencia que va de 6 a 8 por 1,000 recién nacidos vivos. En nuestro país se calcula que cada año nacen de 12,000 a 16,000 niños con algún tipo de malformación cardiaca. En los pacientes no corregidos con cortocircuitos de izquierda a derecha el aumento de la presión pulmonar provoca incremento en las resistencias pulmonares y desencadena una disfunción endotelial y remodelación vascular, consecuencia de alteraciones en los mediadores vasoactivos que provocan vasoconstricción, inflamación, trombosis, proliferación y apoptosis celular así como fibrosis. Como consecuencia hay un incremento progresivo de las resistencias pulmonares y de la presión del ventrículo derecho. Finalmente ocurre que el flujo sanguíneo se invierte y se desarrolla el síndrome de Eisenmenger, la forma más avanzada de hipertensión arterial pulmonar consecutiva a CC. La frecuencia de hipertensión arterial pulmonar secundaria a CC ha disminuido en países desarrollados no así en países en vías de desarrollo tanto por un diagnóstico tardío como por falta de infraestructura hospitalaria o de recursos humanos para la atención de los pacientes portadores de CC. Con el advenimiento del tratamiento farmacológico para la hipertensión arterial pulmonar, se han vislumbrado nuevas oportunidades terapéuticas, siendo cada vez más cotidiano que se sumen al tratamiento intervencionista o quirúrgico en pacientes con hipertensión arterial pulmonar secundaria a CC. Se requiere conocer los factores fisiopatológicos involucrados así como llevar a cabo una cuidadosa evaluación para definir la mejor estrategia terapéutica.

Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy.

[Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome]. / Hipertensión pulmonar asociada a cardiopatías congénitas y síndrome de Eisenmenger.

Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy.

Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies.

The presence of pulmonary arterial hypertension (PAH) increases morbidity and reduces survival in patients with congenital heart disease (CHD). PAH-CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of PAH-specific therapy in the PAH-CHD population, but despite recent advances mortality rates remain relatively high. In the last years, an increasing focus has been placed on patients with PAH-CHD and net left-to-right shunt. Currently, there are limited data to guide the management of these patients and uncertainty on the cut-off values for eventual defect closure. Pregnancy conveys significant risks in PAH-CHD patients: appropriate counselling and care, including psychological support and a multidisciplinary team, should be part of the routine management of women with PAH-CHD of reproductive age. Some subgroups, such as patients with Down's syndrome, Fontan circulation and 'segmental' pulmonary hypertension, present particular challenges in terms of management and therapy. The current review focuses on contemporary treatment strategies in PAH-CHD patients with particular emphasis on challenging patient groups and conditions.

Survey of the current status and management of Eisenmenger syndrome: a Japanese nationwide survey.

BACKGROUND: The management of Eisenmenger syndrome (ES) has dramatically changed since the advent of disease-targeted therapy (DTT). However, guidelines for ES management, including DTT, have not been established. We aimed to clarify the current incidence, underlying disease, and management of ES in Japan, using a nationwide survey. METHODS: A written questionnaire was sent to members of the Japanese Society for Adult Congenital Heart Disease, through which information was obtained from 86 institutions. RESULTS: A total of 251 patients with ES (80.5% cases≥20 years of age) were followed as of February 2012; DTT was performed in 124 (49.4%) patients. Unrepaired simple anatomy was reported as an underlying condition in 165 patients (65.7%). Among patients with ES, 55 (21.9%), 128 (51%), 53 (21.1%), and 12 (4.8%) were classified into functional classes I, II, III, and IV, respectively. DTT was routinely performed at 52 (60.5%) institutions, but there were variations in the DTT therapeutic strategy at these institutions. Combined therapy was more often used than monotherapy; an endothelin receptor antagonist was the most frequently prescribed medication. There were institutional differences regarding heart failure treatment and indications for anticoagulation. Digitalis and angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers were widely used, but beta-blockers were infrequently used to manage heart failure. CONCLUSIONS: This survey describes the current status, including prevalence and underlying disease, and variations in the practical management of ES in Japan. The results will help in the creation of future guidelines for ES management.

[Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease].

OBJECTIVE: To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD). METHODS: Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart catheterization were enrolled from 31 clinical centers in China during the period from May 2007 to October 2010. Age, gender, body mass index (BMI), symptoms and signs, World Health Organization functional class (WHO-FC) of pulmonary hypertension, six-minute walk distance (6MWD) and hemodynamics were recorded. All the above indices were analyzed and compared. RESULTS: A total of 224 patients with Eisenmenger syndrome with 3 kinds of CHD were enrolled, including atrial septal defect (n = 67), ventricular septal defect (n = 104) and patent ductus arteriosus (n = 53). Among them, there were 67 males (29.9%) and 157 females (70.1%) with a mean age of (29.6 ± 9.9) years (range: 15-63). Mean BMI was (19.9 ± 4.0) kg/m(2) and mean 6MWD (371 ± 75) m. The majority of patients were in WHO-FC II (n = 158, 70.5%) and III (n = 64, 28.6%). Electrocardiogram of 77.2% of them indicated hypertrophic right ventricle. Mean right atrial pressure was (8.9 ± 5.7) mm Hg (1 mm Hg = 0.133 kPa), mean pulmonary arterial pressure (mPAP) (77.2 ± 19.1) mm Hg, cardiac index (3.03 ± 1.35) L·min(-1) · m(-2) and pulmonary vascular resistance (PVR) (1621 ± 887) dyn · s · cm(-5). CONCLUSIONS: The majority of patients with Eisenmenger syndrome with different types of CHD are young females and ventricular septal defect is the most frequent underlying cause. The deterioration of heart function in patients with Eisenmenger syndrome is non-parallel to mPAP and PVR in CHD.

First successful heart-lung transplantation in Japan: report of a case.

Although legislation regarding organ transplantation from brain-dead donors in Japan was approved in 1997, no heart-lung transplantation procedures were performed for some time. Herein, we report the first case of successful heart-lung transplantation in Japan. The recipient was a 35-year-old man with Eisenmenger syndrome, caused by a double outlet right ventricle, who had been suffering hemoptysis and continued NYHA class 3-4 symptoms for 66 months since being registered on the waiting list. He underwent heart-lung transplantation from a 30-year-old donor. The organ ischemic time was 247 min, the postoperative course was uneventful, and the patient was discharged 66 days after the transplant surgery.

Eisenmenger syndrome: recent advances in pharmacotherapy.

Over the last decade advanced therapies for the management of pulmonary arterial hypertension have been introduced. These agents have also been effective in reducing pulmonary vascular resistance in patients with Eisenmenger syndrome. Specific guidelines focusing on modern therapies for Eisenmenger syndrome however do not exist to date. More recently, clinical trials in patients with Eisenmenger syndrome demonstrated a significant clinical improvement with favorable safety and tolerability profile. This review aims to summarize newly reported pharmacological agents used in patients with Eisenmenger syndrome.

Efficacy and safety of bosentan in adults with simple and complex Eisenmenger's syndrome.

BACKGROUND: Eisenmenger's syndrome (ES) is associated with decreased longevity and reduced functional capacity. Targeted pharmacologic therapies improve functional capacity and survival in these patients. We sought to compare the response of patients with simple vs. complex ES following initiation of bosentan. METHODS: ES patients with a history of bosentan use were identified by chart review. Simple ES was defined as ES associated with atrial septal defect, ventricular septal defect, or patent ductus arteriosus. Complex ES consisted of patients with truncus arteriosus and single ventricle congenital heart disease. Six-minute walking distance (6MWD), maximal oxygen consumption (VO(2) max), brain natriuretic peptide (BNP), and resting oxygen saturation were compared between simple and complex ES patients before and after bosentan treatment. RESULTS: Twenty-four patients were included (11 simple, 13 complex). Resting oxygen saturation, 6MWD, VO(2) max, and BNP were not significantly different between the two groups prior to bosentan initiation. Ten patients received bosentan monotherapy, and bosentan was used in combination with sildenafil in 13 (five simple, eight complex). One patient received bosentan with iloprost. Mean duration of therapy was 38 ± 14 months in the simple group and 40 ± 8.1 months in the complex group (P= NS). Posttreatment, 6MWD increased from 274 ± 135 m to 326 ± 106 m in simple ES patients (P= .32). 6MWD in patients with complex ES increased from 332 ± 51 m to 364 ± 109 (P= .028). VO(2) max improved from 13.4 ± 3.8 to 17 ± 6 (P= .54) in the simple group, while VO(2) max in the complex group improved from 12.7 ± 2.3 to 15.5 ± 2.2 (P= .17). There was minimal change in BNP or resting oxygen saturation between the groups. CONCLUSIONS: Treatment with bosentan is both safe and effective in patients with both simple and complex forms of ES.