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Dramatic advances in the diagnosis and treatment of congenital heart disease (CHD), the most common inborn defect, has resulted in a growing population of adults with CHD. Eisenmenger syndrome (ES) represents the extreme form of pulmonary arterial hypertension associated with CHD, characterized by markedly increased pulmonary vascular resistance with consequently reversed or bidirectional shunting. While ES is a direct consequence of a heart defect, it is a fundamentally multisystem syndrome with wide-ranging clinical manifestations. The introduction of targeted pulmonary hypertension therapies aimed has subtly shifted clinical focus from preventing iatrogenic and other adverse events toward cautious therapeutic activism.

Guidelines and Expert Consensus Documents summarise and evaluate all currently available evidence on a particular issue with the aim to assist physicians in selecting the best management strategies for a typical patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk/benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks. The legal implications of medical guidelines have been discussed previously. A great number of Guidelines and Expert Consensus Documents have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organisations. Because of the impact on clinical practice, quality criteria for development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines and Expert Consensus Documents can be found on the ESC website (http://www.escardio.org/knowledge/guidelines). In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. Unpublished clinical trial results are not taken into account. A critical evaluation of diagnostic and therapeutic procedures is performed including assessment of the risk/benefit ratio. Estimates of expected health outcomes for larger societies are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to predefined scales, as outlined in tables 1⇓ and 2⇓. The experts of the writing panels have provided disclosure statements of all relationships they may have which might be perceived as real or potential sources of conflicts of interest. These disclosure forms are kept on file at the European Heart House, headquarters of the ESC. Any changes in conflict of interest that arise during the writing period must …

Guidelines and Expert Consensus Documents summarize and evaluate all currently available evidence on a particular issue with the aim to assist physicians in selecting the best management strategies for a typical patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk/benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks. The legal implications of medical guidelines have been discussed previously. A great number of Guidelines and Expert Consensus Documents have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organizations. Because of the impact on clinical practice, quality criteria for development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines and Expert Consensus Documents can be found on the ESC website (http://www.escardio.org/knowledge/guidelines). In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. Unpublished clinical trial results are not taken into account. A critical evaluation of diagnostic and therapeutic procedures is performed including assessment of the risk/benefit ratio. Estimates of expected health outcomes for larger societies are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to predefined scales, as outlined in Tables 1 and 2 . View this table: Table 1 Classes of recommendations View this table: Table 2 Levels of evidence The experts of the writing panels have provided disclosure statements of all relationships they may have which might be perceived as real or potential sources of conflicts of interest. These disclosure forms are kept on file at the European Heart House, headquarters of the ESC. Any changes in conflict of interest that arise …

Objective: To evaluate whether a trial of inhaled nitric oxide (NO) differentiates reversible pulmonary vasoconstriction from fixed anatomic obstruction to pulmonary blood flow after surgery for congenital heart disease in patients at risk for pulmonary hypertension.¶Design: Prospective cohort study.¶Setting: Tertiary care children's hospital.¶Patients: 15 neonate and infants with elevated pulmonary artery or right ventricular pressure or with clinical signs suggestive of high pulmonary vascular resistance in the early postoperative period following repair of congenital heart disease.¶Intervention: 30-min trial of 40 ppm inhaled NO.¶Results: 5 patients responded to inhaled NO, 2 patients were weaned from extracorporeal support with NO. Four were maintained on continuous inhaled NO for 3 to 5 days. All the responders survived. Ten patients did not respond to NO. An important anatomic obstruction was found with echocardiography or angiography in all 10 patients. Reintervention was performed in 6/10 (4 stent placement, 1 balloon angioplasty of pulmonary arteries and 1 revision of systemic to pulmonary shunt). Six of the nonresponders died.¶Conclusion: A trial of inhaled NO after cardiac surgery in neonates and infants may be useful to differentiate reversible pulmonary vasoconstriction from fixed anatomic obstruction and may provide useful information if temporary support with extracorporeal membrane oxygenation is considered. Failure to respond to inhaled NO should prompt further investigations to rule out a residual obstruction.