An Unusual Cause of Irritability in a Single Ventricle Patient after Bidirectional Glenn Shunt

The differential diagnosis for irritability in children is broad.In patients with congenital heart disease,one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure(CVP).In patients with single-ventricle physiology,the second stage of palliation includes bidirectional Glenn,which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle.Typically,early in the post-operative period,patients may experience a headache due to the acute increase in CVP,and symptoms improve over time.Idiopathic intracranial hypertension(IIH),also known as pseudotumor cerebri,is a rare neurologic disorder in children,characterized by raised intracranial pressure(ICP)in the absence of brain parenchymal lesions or cerebrospinal fluid(CSF)abnormalities.While the pathogenesis of IIH is unknown,early recognition and treatment of IIH are important to prevent permanent vision loss.There are only rare reports of IIH in patients with Fontan circulation.To our knowledge,we report the first case of IIH in a 2-year-old female after bidirectional Glenn.

The Glenn Shunt Revisited, A Single Center Registry in Ain Shams University Cardiology Department

Background:Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is,patients unsuitable for biventricular repair.In our country they are performed at an older age than that recommended by the literature.So,we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity.Methods:This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals.History taking including NYHA class and physical activity,clinical examination,six-minutes-walk test,laboratory investigations,full echocardiographic assessment were done for all patients.Some patients needed invasive cardiac catheterization.Results:Our registry included 178 patients who underwent bidirectional Glenn procedure referred for follow up in Ain Shams university hospital from January 2019 till July 2020.The mean age of the registry was 18.7±8.26 years(range between 5 and 37 years),85 males(48%)and 93 females(52%).Regarding the basic anatomy,double outlet right ventricle with hypoplastic left ventricle was the commonest.Furthermore,our descriptive study confirmed many characteristic similarities between our patients and patients in developing countries.Our patients underwent bidirectional Glenn shunt at a median age of 6 years which is considered a relatively old age but similar to other studies that have been made in developing countries like Pakistan,India and Iran.There is a significant delay in the operation in Egyptian patients due to lack of patients’awareness,few numbers of primary health care facilities and high economic burden.Conclusion:Although the presence of slight systemic desaturation,our study demonstrated a satisfactory functional capacity among our patients;thus,bidirectional Glenn can be considered an acceptable definite univentricular repair in patients with late presentation.

Cardiac catheterization and comprehensive clinical evaluation after bidirectional Glenn shunt surgery in 60 patients with complex congenital heart disease

Background The bidirectional Glenn shunt surgery is a palliative procedure for patients with complex congenital heart disease（CHD） who are not suitable for biventricular repair in early life. There is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics that could be used as a reference for patients＇ follow-on management. Methods Sixty CHD patients, 44 male and 16 female, with bidirectional Glenn shunt surgery and cardiac catheterization were enrolled at our hospital between January 2014 and December 2016. Pre-and post Glenn shunt percutaneous oxygen saturation（SpO_2）, 6-minute walk test（6 MWT）, superior vena cava pressure（SVCP）, pulmonary arterial pressure（PAP）, pulmonary capillary wedge pressure（PCWP）, pulmonary vascular resistance（PVR）, small pulmonary vascular resistance（s PVR） were measured. Pre-and post-total cavopulmonary connection（TCPC） SpO_2, and in-hospital complications were monitored. The optimal hemodynamic cutoff values for TCPC patient selection were estimated by receive operating characteristic（ROC） curve analysis. Results SpO_2 was significantly increased by bidirectional Glenn shunt surgery（75.42 ± 9.62% to 86.98 ± 7.63%, P 〈 0.001） from 82.70 ± 5.99% to 95.00 ±4.07% in the 47 patients with TCPC. Forty-two patients completed the 6 MWT with a mean distance of 362.7 ±75.0 m and a SpO_2 decrease from 81.80 ± 7.84% to 67.59 ± 1.82%（P 〈 0.001）. The △SpO_2 and 6-minute walk distance（6 MWD） in the 32 who underwent TCPC and ten of them did not reach statistical significance（17.22 ±13.82% vs. 13.87 ± 8.74%, P = 0.08 and 358.88 ± 78.97 m vs. 374.80 ± 62.55 m, P = 0.564]. After cardiac catheterization, 47 patients were selected for TCPC. The right pulmonary artery systolic pressure（s RPAP）, mean right pulmonary artery pressure（m RPAP）, mean left pulmonary artery pressure（m LPAP）, PVR, and s PVR were significantly lower in the TCPC group than in the non-TCPC group. The differences in superior vena cava systolic blood pressure（s SVCP）, mean superior vena cava pressure（m SVCP）, and left pulmonary artery systolic pressure（s LPAP） were not significant. The optimal cutoff values for TCPC were s SVCP ≤ 20 mm Hg（P = 0.025）,s RPAP ≤ 22 mm Hg（P = 0.0001, mRPAP ≤ 13 mm Hg（P =0.003）, s LPAP ≤ 27 mm Hg（P =0.03）, m LPAP ≤ 11 mm Hg（P = 0.01）, PVR ≤ 4.3 Wood U/m^2（P 〈0.0001） and were significantly associated with TCPC selection,except for m SVCP ≤ 19 mm Hg（P = 0.06） and s PVR ≤ 2.0 wood U/m^2（P = 0.0531）. One patient died because of low cardiac output after TCPC. In-hospital mortality was 2.1%. Conclusion The SpO_2 can be significantly improved after bidirectional Glenn shunt and TCPC surgery. The 6 MWT is an index of activity tolerance prior toTCPC. Hemodynamic values of s SVCP ≤ 20 mm Hg, s RPAP ≤ 22 mm Hg, m RPAP ≤ 13 mm Hg, s LPAP ≤ 27 mm Hg, m LPAP ≤ 11 mm Hg, and PVR ≤ 4.3 Wood U/m^2 can help identify post Glenn-shunt patients indicated for TCPC.

A successful case of bidirectional Glenn Shunt for adult single ventricular disease

INTRODUCTION Single ventricle is one type of infrequent and complex congenital heart disease. Bidi- rectional Glenn shunt operation is becoming the most common and effective palliative op- eration of single ventricle to improve the symptoms before modified Fontan operation. However, Bidirectional Glenn shunt opera- tion is rarely applied to the adult patient （more than 20 years old） with single ventri- cle because of the low survival rate1. In this report, we presented a six years following-up study of the Bidirectional Glenn shunt in a 37-year-old female patient with single ventricle.