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Tomasz Mroczek

Bio: Tomasz Mroczek is an academic researcher from Jagiellonian University. The author has contributed to research in topics: Hypoplastic left heart syndrome & Norwood procedure. The author has an hindex of 10, co-authored 42 publications receiving 440 citations. Previous affiliations of Tomasz Mroczek include Jagiellonian University Medical College & Boston Children's Hospital.

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Journal ArticleDOI
TL;DR: A stable haemodynamic status due to independent coronary perfusion, higher diastolic and lower pulse pressure is the most advantageous effect of RV-PA, resulting in a lower mortality and morbidity after NP.
Abstract: Objective: The aim of this study was to assess changes in early and late haemodynamic status after the Norwood procedure (NP), caused by the implementation of right ventricle-to-pulmonary artery shunt (RV-PA). Methods: A consecutive series of 68 children with hypoplastic left heart syndrome underwent NP: Group 1 (n ¼ 31) with the application of a modified Blalock-Taussig shunt and Group 2 (n ¼ 37) with RV-PA. Haemodynamic data from the early postoperative period (72 h after the operation) and cardiac catheterisation data, as well as blood tests before the hemi-Fontan procedure (HF) were analysed. Univariate (x 2 test, Mann ‐ Whitney’s and Student’s t-tests) and multiple regression analysis were carried out. Results: In Group 1, circulatory collapse requiring resuscitation occurred in 15 (48.4%) children, within 72 h after the procedure. The resuscitation was unsuccessful in nine (29%) cases. The operative mortality (30 days) was 35%. In Group 2, two (5%) children died within the early and two (5%) within the late postoperative period. The postoperative course in the remaining children from Group 2 was uneventful. In Group 2 there was a significantly higher mean diastolic pressure after NP (P , 0:05). The arterial pulse pressure after NP was significantly lower in Group 2 (P , 0:05). Before HF, the application of RV-PA was associated with a lower Qp:Qs ratio (P ¼ 0:020), lower aortic pulse pressure (P ¼ 0:004) and lower aortic oxygen saturation (P ¼ 0:039). Conclusions: A stable haemodynamic status due to independent coronary perfusion, higher diastolic and lower pulse pressure is the most advantageous effect of RV-PA, resulting in a lower mortality and morbidity after NP. A lower Qp:Qs ratio eliminates the danger of the ventricular volume overload and ensures good conditions for the development of the pulmonary circulation before HF. q 2003 Elsevier Science B.V. All rights reserved.

98 citations

Journal ArticleDOI
TL;DR: It is indicated that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group.
Abstract: We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations. The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients. These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.

73 citations

Journal ArticleDOI
TL;DR: The effectiveness of emergency extracorporeal life support in treating life-threatening simultaneous propranolol and verapamil intoxication is shown and should be considered early in cases of near-fatal intoxications with cardiodepressive drugs.
Abstract: Combined poisoning with calcium-channel blockers and beta-blockers is usually associated with severe heart failure. This report shows the effectiveness of emergency extracorporeal life support in treating life-threatening simultaneous propranolol and verapamil intoxication. A 15-year-old girl presented in cardiogenic shock after alcohol consumption and a propranolol and verapamil overdose; plasma concentrations: propranolol, 0.53 m/mL; verapamil, 1.06 mg/mL. She was successfully resuscitated with extracorporeal life support. Therapeutic plasma exchange was initiated. Extracorporeal support was discontinued 70 hours later. The patient made a full recovery. Simultaneous verapamil and propranolol overdoses can cause severe hemodynamic compromise and arrest of electrical and mechanical function of the heart. Emergency extracorporeal life support can successfully maintain vital organ blood flow and allows time for drug metabolism, redistribution, and removal. Therapeutic plasma exchange may reduce the time of emergency extracorporeal life support. Emergency extracorporeal life support should be considered early in cases of near-fatal intoxications with cardiodepressive drugs.

69 citations

Journal ArticleDOI
TL;DR: The Norwood procedure with the RV-PA shunt provides satisfactory late hemodynamics and improves the development of the pulmonary arteries and children with hypoplastic left heart syndrome subjected to this new method of palliation are good candidates for the hemi-Fontan procedure.
Abstract: Objective: The advantageous effect of right ventricle-to-pulmonary artery shunt (RV‐PA) on the early postoperative hemodynamics in the Norwood procedure for hypoplastic left heart syndrome (HLHS) is well known. Numerous controversies still exist with respect to the late consequences of this new palliation method in preparation for the second stage procedure. Methods: Between September 1997 and September 2004, a consecutive series of 78 children with HLHS from a single institution underwent the hemi-Fontan procedure: Group 1 (nZ27) after Blalock‐Taussig shunt (BT), and Group 2 (nZ51) after RV‐PA. Hemodynamic, echocardiographic and clinical perioperative data were analyzed. Results: There were no significant differences in the age and operative weight (Group 1: 6.9G1.04 months, 6.22G0.99 kg; Group 2: 6.57G1.12 months, 6.36G0.86 kg). Children after RV‐PA were characterized by a significantly higher preoperative hematocrit value (PZ0.014), lower aortic and superior vena cava oxygen blood saturation (P!0.001, PZ0.024), severe right ventricle hypertrophy more rarely diagnosed in echocardiography (P!0.004), lower Qp:Qs ratio (PZ0.011), larger right (PZ0.001) and left (PZ0.006) pulmonary artery index and a shorter intensive care unit stay after the hemi-Fontan procedure (PZ0.004). Conclusions: The Norwood procedure with the RV‐PA shunt provides satisfactory late hemodynamics and improves the development of the pulmonary arteries. Children with hypoplastic left heart syndrome subjected to this new method of palliation are good candidates for the hemi-Fontan procedure. Q 2005 Elsevier B.V. All rights reserved.

43 citations

Journal ArticleDOI
TL;DR: Evaluated coagulation profile in the early postoperative period after hemi-Fontan and Fontan procedures with relationship to liver function and hemodynamic variables could contribute to postoperative thromboembolic complications.
Abstract: Introduction: The causes of coagulation abnormalities and thromboembolic complications during staged Fontan approach in patients with single ventricle remain unclear. This study was designed to evaluate the coagulation profile in the early postoperative period after hemi-Fontan and Fontan procedures with relationship to liver function and hemodynamic variables. Materials and methods: The prospective study on 43 patients after hemi-Fontan (group 1) and 37 patients after Fontan procedure (group 2) was carried out. Coagulation profile (factor VII, factor VIII, ATIII, fibrinogen, prothrombin), liver function (total serum protein, albumin, AST, ALT, bilirubin), and hemodynamic variables were assessed on postoperative day 1 and 5 and compared to preoperative measures. Results: Factor VIII concentration was significantly higher on first postoperative day in both groups. On postoperative day 5 the concentration of factor VIII was significantly decreased in group 1 whereas constant in group 2. The concentration of factor VII, ATIII, fibrinogen, and prothrombin was significantly decreased on first and increased on fifth postoperative day after both hemi-Fontan and Fontan procedures. The increase in bilirubin concentration was more distinctive after Fontan operation (p = 0.003) with lower AST in this group (p < 0.0001). The single ventricle function, pO2 and central venous pressure had significant influence on factor VIII (p = 0.034), factor VII (p = 0.012), ATIII (p = 0.006), and prothrombin (p = 0.024) concentrations in group 2 with no significant influence in group 1. Conclusions: The distinctive causes of coagulation abnormalities during staged Fontan approach are hemodynamic changes and temporary liver dysfunction. Elevated concentration of factor VIII and significant influence of hemodynamics on coagulation profile could contribute to postoperative thromboembolic complications. # 2007 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

42 citations


Cited by
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Journal ArticleDOI
TL;DR: The estimate of Down syndrome prevalence is roughly 25%-40% lower than estimates based solely on current birth prevalence, and can be considered a starting point for facilitating policy and services planning for persons with Down syndrome.

315 citations

Journal ArticleDOI
TL;DR: Surgical strategies based on staged procedures are discussed, with the right ventricle supporting both systemic and pulmonary circulation, and other management options, such as neonatal transplantation and the recent innovation of hybrid techniques are discussed.

262 citations

Journal ArticleDOI
TL;DR: Thrombosis has long been recognized as a potentially life-threatening complication in children with congenital heart disease (CHD), children with acquired heart disease, and in adults with CHD.
Abstract: Thrombosis has long been recognized as a potentially life-threatening complication in children with congenital heart disease (CHD), children with acquired heart disease, and in adults with CHD. High-risk groups include patients with shunt- dependent single ventricles (shunt thrombosis, 8%–12%; 4%

256 citations

Journal ArticleDOI
TL;DR: Pulse oximetry is a promising alternative newborn screening strategy but further evaluation is needed to obtain more precise estimates of test performance and to inform optimal timing, diagnostic and management strategies.
Abstract: OBJECTIVES: To provide evidence to inform policy decisions about the most appropriate newborn screening strategy for congenital heart defects, identifying priorities for future research that might reduce important uncertainties in the evidence base for such decisions DATA SOURCES: Electronic databases Groups of parents and health professionals REVIEW METHODS: A systematic review of the published medical literature concerning outcomes for children with congenital heart defects was carried out A decision analytic model was developed to assess the cost-effectiveness of alternative screening strategies for congenital heart defects relevant to the UK A further study was then carried out using a self-administered anonymous questionnaire to explore the perspectives of parents and health professionals towards the quality of life of children with congenital heart defects The findings from a structured review of the medical literature regarding parental experiences were linked with those from a focus group of parents of children with congenital heart defects RESULTS: Current newborn screening policy comprises a clinical examination at birth and 6 weeks, with specific cardiac investigations for specified high-risk children Routine data are lacking, but under half of affected babies, not previously identified antenatally or because of symptoms, are identified by current newborn screening There is evidence that screen-positive infants do not receive timely management Pulse oximetry and echocardiography, in addition to clinical examination, are alternative newborn screening strategies but their cost-effectiveness has not been adequately evaluated in a UK setting In a population of 100,000 live-born infants, the model predicts 121 infants with life-threatening congenital heart defects undiagnosed at screening, of whom 82 (68%) and 83 (69%) are detected by pulse oximetry and screening echocardiography, respectively, but only 39 (32%) by clinical examination alone Of these, 71, 71 and 34, respectively, receive a timely diagnosis The model predicts 46 (05%) false-positive screening diagnoses per 100,000 infants with clinical examination, 1168 (13%) with pulse oximetry and 4857 (54%) with screening echocardiography The latter includes infants with clinically non-significant defects Total programme costs are predicted of pound 300,000 for clinical examination, pound 480,000 for pulse oximetry and pound 354 million for screening echocardiography The additional cost per additional timely diagnosis of life-threatening congenital heart defects ranges from pound 4900 for pulse oximetry to pound 45 million for screening echocardiography Including clinically significant congenital heart defects gives an additional cost per additional diagnosis of pound 1500 for pulse oximetry and pound 36,000 for screening echocardiography Key determinants for cost-effectiveness are detection rates for pulse oximetry and screening echocardiography Parents and health professionals place similar values on the quality of life outcomes of children with congenital heart defects and both are more averse to neurological than to cardiac disability Adverse psychosocial effects for parents are focused around poor management and/or false test results CONCLUSIONS: Early detection through newborn screening potentially can improve the outcome of congenital heart defects; however the current programme performs poorly, and lacks monitoring of quality assurance, performance management and longer term outcomes Pulse oximetry is a promising alternative newborn screening strategy but further evaluation is needed to obtain more precise estimates of test performance and to inform optimal timing, diagnostic and management strategies Although screening echocardiography is associated with the highest detection rate, it is the most costly strategy and has a 5% false-positive rate Improving antenatal detection of congenital heart defects increases the cost per timely postnatal diagnosis afforded by any newborn screening strategy but does not alter the relative effects of the strategies An improvement of timely management of screen positive infants is essential Further research is required to refine the detection rate and other aspects of pulse oximetry, to evaluate antenatal screening strategies more directly, and to investigate the psychosocial effects of newborn screening for congenital heart defects

237 citations