Miyahara em et al /em . as well as the linked ventricular quantity overload in one ventricle physiology. Impaired ventricular function comes with an adverse impact 6-Maleimido-1-hexanol on both early and past due outcome throughout Fontan conclusion [2]. As a result, we prepared the mixed therapy of early bidirectional Glenn process of the ventricular quantity decrease and early treatment including -blocker. Though it had bought out 2 years to acquire enough ventricular function (Fig. ?(Fig.2),2), this plan you could end up an effective Fontan conclusion with satisfactory result. Avoidance of embolic problem can be an essential requirement of administration also. Prophylactic anticoagulation and antiplatelet therapy is preferred for everyone sufferers with LVNC, if thrombus or atrial fibrillation is available [1]. Systolic dysfunction in LVNC is certainly regarded as connected with subendocardial microcirculatory and hypoperfusion dysfunction. Chin em et al /em . [3] recommended that subendocardial perfusion might be abnormal in LVNC despite the absence of coronary artery disease. Miyahara em et al /em . emphasize the importance of keeping pulsatile blood flow during CPB and avoidance of cardiac arrest to maintain microcirculation [4]. Secondly, a fenestration was added with the aim of increasing the ventricular filling volume and the following cardiac output, which could be effective under the significant influence of diastolic dysfunction associated with LVNC. However, the indication of fenestration in this case is controversial, because increase in oxygen saturation is the one of the reasons for completion of TCPC especially in reduced LV contractility. It is unclear which factor, the increased preload or saturation, contributes to the Fontan circulation. However, as one of the answers, we believe that this staged Fontan completion, with a fenestrated Fontan operation located between the Glenn circulation and the non-fenestrated Fontan circulation, could result in a satisfactory surgical outcome. Conflict of interest: none declared. REFERENCES 1. Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation 2004;109:2965C71. [PubMed] [Google Scholar] 2. Hosein RB, Clarke AJ, McGuirk SP, Griselli M, Stumper O, De Giovanni JV et al. . Factors influencing early and late outcome following the Fontan procedure in the current era. The Two Commandments? Eur J Cardiothorac Surg 2007;31:344C53. [PubMed] [Google Scholar] 3. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990;82:507C13. [PubMed] [Google Scholar] 4. Miyahara Y, Kasahara S, Ishino K, Sakurai S, Sano S. Successful Fontan completion in a patient with noncompaction myocardium. J Thorac Cardiovasc Surg 2010;139:e85C7. [PubMed] [Google Scholar] 5. McMahon CJ, Nolke L. Successful palliation of a child Rabbit Polyclonal to RAB3IP with left ventricular noncompaction cardiomyopathy and tricuspid atresia to Fontan procedure. Ann Thorac Surg 2014;98:719C21. [PubMed] [Google Scholar].[3] suggested that subendocardial perfusion might 6-Maleimido-1-hexanol be abnormal in LVNC despite the absence of coronary artery disease. in this special setting, it is important to improve and maintain cardiac function for both the LVNC condition 6-Maleimido-1-hexanol and Fontan circulation. Standard medical treatment such as with -blocker is indicated for a patient with LVNC whose LVEF is impaired. One of the mechanisms of action of -blockers is to prevent and reverse adrenergically mediated intrinsic myocardial dysfunction and remodelling. On the other hand, systemicCpulmonary shunt might result in the impaired cardiac function by means of the pulmonary and the associated ventricular volume overload in single ventricle physiology. Impaired ventricular function has an adverse influence on both early and late outcome in the course of Fontan completion [2]. Therefore, we planned the combined therapy of early bidirectional Glenn procedure for the ventricular volume reduction and early medical treatment including -blocker. Although it had taken over 2 years to obtain sufficient ventricular function (Fig. ?(Fig.2),2), this strategy could result in a successful Fontan completion with satisfactory result. Prevention of embolic complication is also an important aspect of management. Prophylactic antiplatelet and anticoagulation therapy is recommended for all patients with LVNC, whether or not thrombus or atrial fibrillation is found [1]. Systolic dysfunction in LVNC is thought to be associated with subendocardial hypoperfusion and microcirculatory dysfunction. Chin em et al /em . [3] suggested that subendocardial perfusion might be abnormal in LVNC despite the absence of coronary artery disease. Miyahara em et al /em . emphasize the importance of keeping pulsatile blood flow during CPB and avoidance of cardiac arrest to maintain microcirculation [4]. Secondly, a fenestration was added with the aim of increasing the ventricular filling volume and the following cardiac output, which could be effective under the significant influence of diastolic dysfunction associated with LVNC. However, the indication of fenestration in this case is controversial, because increase in oxygen saturation is the one of the reasons for completion of TCPC especially in reduced LV contractility. It is unclear 6-Maleimido-1-hexanol which factor, the increased preload or saturation, contributes to the Fontan circulation. However, as one of the answers, we believe that this staged Fontan completion, with a fenestrated Fontan operation located between the Glenn circulation and the non-fenestrated Fontan circulation, could result in a satisfactory surgical outcome. Conflict of interest: none declared. REFERENCES 1. Weiford BC, Subbarao VD, Mulhern KM. Noncompaction of the ventricular myocardium. Circulation 2004;109:2965C71. [PubMed] [Google Scholar] 2. Hosein RB, Clarke AJ, McGuirk SP, Griselli M, Stumper O, De Giovanni JV et al. . Factors influencing early and late outcome following the Fontan procedure in the current era. The Two Commandments? Eur J Cardiothorac Surg 2007;31:344C53. [PubMed] [Google Scholar] 3. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 1990;82:507C13. [PubMed] [Google Scholar] 4. Miyahara Y, Kasahara S, Ishino K, Sakurai S, Sano S. Successful Fontan completion in a patient with noncompaction myocardium. J Thorac Cardiovasc Surg 2010;139:e85C7. [PubMed] [Google Scholar] 6-Maleimido-1-hexanol 5. McMahon CJ, Nolke L. Successful palliation of a child with left ventricular noncompaction cardiomyopathy and tricuspid atresia to Fontan procedure. Ann Thorac Surg 2014;98:719C21. [PubMed] [Google Scholar].