Prognosis for patients with Eisenmenger syndrome of various aetiology

Saha, Anup Kumar; Balakrishnan, K.G.; Jaiswal, Praveen Kumar; Venkitachalam, C.G.; Tharakan, Jaganmohan; Titus, Thomas; Kutty, Raman

doi:10.1016/0167-5273(94)90166-x

Cited by 136 publications

(83 citation statements)

References 9 publications

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“…In a similar way, unoperated patients with large, unrestrictive VSDs or ASDs are also at risk (>50% and ∼10% risk, respectively). [4][5][6][7][8][9] In addition to the size and type of the defect (for example, posttricuspid vs. pretricuspid communications), late referral, the presence of associated extracardiac syndromes (especially Down syndrome), and the degree of obstruction to the pulmonary venous flow (particularly in complex anomalies) are important risk factors.…”

mentioning

confidence: 99%

Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

et al. 2014

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Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

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confidence: 99%

Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

et al. 2014

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“…In ES patients, higher functional class, a history of arrhythmias, patient age, right ventricular dysfunction, syncope, elevated uric acid levels and the presence of Down's syndrome have been found to be of value in predicting a poor outcome [26,[30][31][32][33][34]. In a very recent study based on data of 181 ES patients, a composite echocardiographic score was also highly predictive of clinical outcome [35].…”

Section: Outcome and Outcome Predictorsmentioning

confidence: 99%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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“…Progressive subpulmonary ventricular failure and premature death are the rule in adults with Eisenmenger syndrome, with immediate causes of death including pulmonary ventricular failure, severe hemoptysis from bronchial artery rupture or pulmonary infarction, complications during pregnancy, and cerebral vascular events, including occlusive strokes, systemic paradoxical embolization, and brain abscesses. 264,491,492 Death during noncardiac surgery also occurs. Poor functional class is a significant predictor of mortality for Eisenmenger patients, as are serological evidence of low systemic organ perfusion, worsened hypoxemia, and LV systemic dysfunction.…”

Section: Eisenmenger Physiologymentioning

confidence: 99%

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease

Warnes¹,

Williams²,

Bashore³

et al. 2008

Circulation

884

386

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Prognosis for patients with Eisenmenger syndrome of various aetiology

Cited by 136 publications

References 9 publications

Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease

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