Andrzej Sysa

Interventions Complementing Surgery as Part of Multistage Treatment for Hypoplastic Left Heart Syndrome: One Center's Experience

Background Interventional procedures often are used as part of multistage treatment for hypoplastic left heart syndrome (HLHS). This study aimed to evaluate the efficacy of interventions in multistage treatment of HLHS. Methods and Results Between 1999 and 2007, 78 interventions were performed for 58 children with HLHS at different stages of surgical treatment. Group 1, involving 30 interventions for 22 patients with postoperative pulmonary arterial stenosis, showed reduced pulmonary artery pressure and increased vessel diameter. Group 2, involving 15 interventions after the Norwood operation for 12 patients with aortic arch obstruction, showed success for 11 patients. Group 3, involving eight balloon angioplasties of narrowed Glenn anastomosis for seven patients, showed abolition of the pressure gradient between the superior vena cava and the right pulmonary artery. Group 4, involving eight interventional closures of a fenestration for eight patients after a Fontan operation, showed increased oxygen saturation for all eight patients. Group 5, involving seven interventions for six patients in whom venovenous collaterals were closed percutaneously, showed improved oxygen saturation. For Group 6, uncommon miscellaneous interventions were used to stabilize the patients’ condition before the next surgical treatment. Conclusions Interventional procedures play an important role during multistage treatment of HLHS. They allow for a reduction in the number of operations or stabilization of the patients’ condition before the next surgical treatment.

Bronchial compression as adverse effect of left pulmonary artery stenting in a patient with hypoplastic left heart syndrome.

A 5-year-old child with hypoplastic left heart syndrome (HLHS) was noted to have left pulmonary artery stenosis after Glenn shunt placement. Therefore, a stent was implanted into the left pulmonary artery to relieve stenosis. After redilatation of the pulmonary artery stent, wheezing, fatigue, asymmetric bronchial sound, and desaturation was noted. Computed tomography, bronchoscopy, and bronchography confirmed critical left main bronchus compression as a consequence of the mass effect of the dilated stent. Balloon plasty of the left bronchus successfully relieved bronchial stenosis. Tracheobronchial obstruction is a possible adverse effect of vascular stenting in children with congenital heart defects. Balloon plasty seems to be a safe and efficient therapeutic option in those cases.

A 10-year single-centre experience in percutaneous interventions for multi-stage treatment of hypoplastic left heart syndrome

OBJECTIVES The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications. BACKGROUND Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment. METHODS Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation. RESULTS Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries. CONCLUSIONS Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

The three-vessel view in the fetal mediastinum in the diagnosis of interrupted aortic arch.

Interruption of the aortic arch (IAA) is difficult to detect and diagnose in utero. However, prenatal diagnosis may be beneficial because IAA is rapidly fatal (median age, 10 d) if left uncorrected. Our objective was to review the direct and indirect echocardiographic markers associated with IAA, focusing on the importance of the three-vessel view (3VV), which is obtained during routine ultrasound examination to rule out malformations. We analyzed the fetal echocardiograms of nine fetuses and compared them with 56 normal controls. In each fetus, there was a large discrepancy between the diameter of the larger, dilated pulmonary artery (PA) and smaller, narrow aortic arch (Ao). The calculated ratio of PA/Ao in fetuses with IAA was 2.6 ± 0.4 compared with 1.1 ± 0.09 in normal controls (p < 0.0001). The calculated ratio of PA/Ao in fetuses with IAA type A was 2.1 ± 0.09 and IAA type B 2.9 ± 0.2 (p = 0.0007). Discrepancy between PA/Ao diameters should raise the suspicion of aortic arch anomalies and a large discrepancy is a nearly pathognomonic sign of IAA type B.

Fracture and dislocation of a pulmonary stent after cardiac massage.

Following a Glenn procedure, a stent was implanted into the pulmonary artery to relieve stenosis. After the procedure, the patient developed bradycardia and became asystolic. Resuscitation was successful following cardiac massage, but subsequent catheterization revealed the stent to be broken in two, with shift of the distal fragment. The two parts were stabilized and connected by implantation of an additional CP stent.

Right ventricular outflow tract giant pseudoaneurysm: percutaneous approach and complications

We report a case of a 19-year-old patient with double outlet right ventricle (RV) and recurrent giant RV outflow tract pseudoaneurysm, after multiple redo surgery. The patient underwent implantation of a 10 mm Amplatzer Septal Occluder to close the pseudoaneurysm. Postinterventional echocardiography revealed dislocation of the device into the cavity of the pseudoaneurysm. Consecutive computed tomography enabled three-dimensional measurements of the pseudoaneurysmand its orifice and resulted in implantation of a 20 mm occluder.

P03.17: Tetralogy of Fallot (ToF) in a series of 40 cases in a fetal cardiology center in Lodz in years 1994–2009

S. Forys1, K. Janiak2, A. Zarkowska2, M. Slodki1, M. Respondek-Liberska1,2, A. Sysa3 1Department of Diagnosis and Prophylaxis of Congenital Malformations, Institute of Polish Mother Memorial Hospital, Lodz, Poland; 2Department of Diagnosis and Prophylaxis of Congenital Malformations, Institute of Polish Mother Memorial Hospital & Medical University, Lodz, Poland; 3Department of Pediatric Cardiology, Institute of Polish Mother Memorial Hospital, Lodz, Poland

Wpływ zabiegu kardiochirurgicznego w krążeniu pozaustrojowym w pierwszym roku życia na dystrybucję populacji limfocytów krwi obwodowej

Wstep Aktywacja nieswoistych mechanizmow ukladu odpornościowego stwierdzana u noworodkow i niemowląt z wrodzoną wadą serca predysponuje do czestszych zakazen i ich szybkiego uogolniania. W trakcie zabiegow kardiochirurgicznych z uzyciem krązenia pozaustrojowego, szczegolnie u najmlodszych dzieci, istnieje potrzeba wykonania cześciowej lub calkowitej tymektomii. Cel Celem pracy byla ocena wplywu zabiegu kardiochirurgicznego z wykorzystaniem krązenia pozaustrojowego wykonywanego w pierwszym roku zycia na dystrybucje populacji limfocytow krwi obwodowej. Material i metody Badaniami objeto 32 dzieci z wrodzonymi wadami serca operowanych w pierwszym roku zycia. Oceniano subpopulacje limfocytow krwi obwodowej za pomocą metody cytometrycznej i testu IMK-Plus. Ponadto u cześci dzieci wykonano badanie komorek pamieci immunologicznej. Oznaczenia wykonywano trzykrotnie: przed zabiegiem kardiochirurgicznym, we wczesnym oraz odleglym okresie pooperacyjnym. Wyniki W kolejnych badaniach wykazano obnizanie sie odsetka limfocytow CD3+ i CD4+ oraz wzrost odsetka limfocytow CD19+ w stosunku do wartości sprzed zabiegu. Istotne statystycznie roznice obserwowano porownując wartości uzyskane z drugiego i trzeciego oznaczenia (CD3+, CD4+) oraz wyjściowe z odleglymi (CD3+, CD4+, CD19+). Średni odsetek komorek CD45CD4RO+ w krotkim okresie pooperacyjnym byl nizszy w porownaniu z wartościami sprzed zabiegu, a w kolejnych miesiącach wzrastal istotnie statystycznie. Wnioski Wyniki dotychczasowych badan potwierdzają, ze zabieg kardiochirurgiczny z zastosowaniem krązenia pozaustrojowego powoduje zmiany odsetkow subpopulacji limfocytow krwi obwodowej oraz komorek pamieci immunologicznej w krotkim, a takze w odleglym okresie pooperacyjnym. Moze to wplywac na funkcjonowanie ukladu odpornościowego tych dzieci zarowno w okresie okolozabiegowym, jak i w dalszym zyciu. Konieczna jest kontynuacja badan w dluzszym okresie pozabiegowym oraz porownanie statusu immunologicznego dzieci operowanych z powodu wrodzonych wad serca z ich zdrowymi rowieśnikami.

OC028: Natural history of fetal Ebstein's anomaly in a referral center in the second half of pregnancy

In postnatal life progression of heart disease has been clearly documented. Natural history studies have been performed and as a consequence, guidelines for interventions (drugs, interventional or surgical procedures) have been developed. Early detection of fetal heart disease, even in the first trimester, is feasible and these fetuses can be followed in-utero for months. Progression of fetal congenital heart disease has been widely documented in case reports and small case series. Any cardiac valve can become more stenotic, even atretic or more incompetent during the course of pregancy, potentially leading to secondary damage to the heart and lungs. Consecutive impaired growth of ventricles and/or vessels may result in a univentricular circulation postnatally. Valve regurgitations have the potential to lead to hypertrophy, congestive heart failure and hydrops. Theoretically, as in postnatal life, timely fetal intracardiac intervention should lead to normalization or improvement of flows, pressures and hemodynamics, thus preventing secondary damage to the fetal heart and organs, congestive heart failure or fetal death. Cardiac lesions, that might profit from timely interventions could be subgroups of: critical valvar aortic stenosis, critical valvar pulmonary stenosis, pulmonary atresia with intact septum or a closed or severely restrictive foramen ovale in cases of left heart obstructive lesions like e.g. hypoplastic left heart syndrome. Successful in-utero dilatation of aortic and pulmonary valve, perforation of valvar pulmonary atresia and atrio-septostomy with ballon dilation of the created ASD have already been reported. As these procedures carry a high risk of technical problems, morbidity and mortality, careful patient selection is crucial.