Primary cardiac tumours: how well can prenatal diagnosis "predict" postnatal outcome?

OBJECTIVE: Primary foetal cardiac tumours are rare congenital malformations. They can cause a flow obstruction, arrhythmias and can lead to cardiac failure, hydrops or death. Postnatal management is based on patient´s clinical and hemodynamic impairment. METHODS: We retrospectively reviewed data from 2009-2019 from our gynaecology clinic and also data regarding postnatal follow-up from our partner paediatric institution. RESULTS: In this period, we diagnosed six cases with foetal cardiac tumours. In four cases, multiple rhabdomyomas were present. Three patients did not have serious complications pre- or postnatally. In one case, tumours were obliterating both the inflow and the outflow of the left ventricle. The child died at three months of age. Tuberous sclerosis was confirmed in all the cases with rhabdomyomas. One child had a fibroma filling the left ventricle. Despite an uneventful prenatal period, the patient got postnatally symptomatic. Tumour was considered inoperable and the child died at the age of five months. In one case a single right ventricular unspecified tumour was diagnosed, without any complications. CONCLUSION: Prognosis closely depends on early diagnosis, clinical manifestations and the possibility of surgical tumour removal if necessary. In confirmed rhabdomyomas, tests for tuberous sclerosis are mandatory (Tab. 1, Fig. 2, Ref. 18). Text in PDF www.elis.sk Keywords: rhabdomyoma, fibroma, prenatal diagnosis, ultrasound, tuberous sclerosis.

Incidence of Diabetes Mellitus After Kidney Transplantation in Slovakia: Multicentric, Prospective Analysis.

BACKGROUND: The incidence rate of post-transplant diabetes mellitus (PTDM) after kidney transplantation (KT) is 5% to 40%. The objective of this analysis was to identify the risk factors of PTDM after KT in the Slovak Republic (SR). METHODS: In the group of 133 patients/non-diabetics, we identified the risk factors of PTDM in the monitored period of 12 months from transplantation. RESULTS: The incidence of PTDM in the SR in 2014 was 38.3%. By logistic regression, we discovered that the age at the time of KT [odds ratio, 1.0885; 95% CI, 1.0222-1.1592; P = .0082], the value of body mass index (BMI) at the time of KT [odds ratio, 1.4606; 95% CI, 1.0099-2.1125; P = .0442], and the value of insulin resistance index (homeostatic model assessment for insulin resistance) at the time of KT [odds ratio, 2.5183; 95% CI, 1.7119-3.4692; P < .0001] represented predictive factors of PTDM. The independent risk factors of PTDM in our group were age at the time of KT of more than 60 years [HR 0.3871; 95% CI 0.1659-1.7767; P = .0281], waist circumference at the time of KT in men more than 94 cm and in women more than 80 cm [HR, 3.4833; 95% CI, 1.2789-9.4878 (P = .0146)], BMI at the time of KT [HR 3.0011; 95% CI 1.0725-8.3977 (P = .0363)], and triacylglycerols at the time of KT more than 1.7 mmol/L [HR, 2.9763; 95% CI, 1.0141-8.7352; P = .0471]. CONCLUSIONS: In the group of Slovak patients after kidney transplantation, the dominating risk factor for PTDM development was insulin resistance prior to KT.

Preoperative NT-proBNP values in patients with atrioventricular septal defect and its role as a predictor of early postoperative course.

OBJECTIVES: Atrioventricular septal (AVSDC) defect presents by excessive pulmonary blood flow and congestive heart failure in children. This study was designed to identify and compare N-terminal pro-brain natriuretic peptide (NT-proBNP) values in complete and incomplete form at the time of surgery, and evaluation of postoperative course. METHODS: The prospective study included thirty-one patients with complete and fifteen with intermediate forms, undergoing surgery in the period from May 2009 to January 2014. Pre- and post-operative variables were statistically analyzed. RESULTS: The median value of NT-proBNP was 1420.0 pg/mL, with elevated levels in complete form (1656.0 vs 488.0 pg/mL, p < 0.001); age and weight with negative correlation. Other preoperative variables (genetic disorder, atrioventricular valve regurgitation, borderline ventricle) were not significant. Higher incidence of postoperative variables was present in complete form, namely mechanical ventilation (p < 0.03), dose and length of vasopressors (p < 0.002), ICU stay (p < 0.04), and complications (p < 0.05). NT-proBNP analysis as a predictor of postoperative course showed an association with longer ventilatory (> 48 hours) and inotropic forms of support, ICU stay, and presence of infection. CONCLUSION: NT-proBNP value might be helpful in the decision-making process of early surgical treatment of complete form of AVSDC, and useful as a predictor of early post-operative course (Tab. 4, Fig. 3, Ref. 14).

Shock--manifestation of pulmonary atresia with aorto-pulmonary collaterals and single ventricle physiology in a 2-day neonate.

BACKGROUND: The combination of pulmonary valve atresia and ventricular septal defect accounts for about 2% of cases of congenital heart disease. Most of the cases have intracardiac anatomy of Tetralogy Fallot and present with cyanosis in neonatal age. OBJECTIVES: To report a case of a newborn with rare combination of pulmonary atresia, ventricular septal defect and single ventricle physiology presenting with shock very early following birth. METHODS AND RESULTS: We describe a newborn infant who developed shock with severe metabolic acidosis and respiratory distress several hours following birth. Cardiac ultrasound showed pulmonary atresia and ventricular septal defect with single ventricle physiology. Cardiac cathetrisation revealed major aorto-pulmonary collaterals with an excessive pulmonary blood flow. Resuscitative measures resulted in hemodynamic stability. However, due to unfavorable prognosis, a decision not to undertake surgical palliation was made. Withdrawal of intensive care led to rapid demise of the patient. CONCLUSIONS: We report a case of a newborn with pulmonary atresia, ventricular septal defect and single ventricle physiology in whom an excessive flow through major aorto-pulmonary collaterals led to shock and death very early following birth (Fig. 1, Ref. 5). Full Text (Free, PDF) www.bmj.sk.

Neonates with left-sided obstructive heart disease: clinical manifestation and management at primary care hospitals.

BACKGROUND: In neonates, left-sided obstructive heart defects are critical diseases requiring early recognition, initial stabilization, and transfer to Cardiac Center. OBJECTIVES: To assess management of these neonates in primary care hospitals. METHODS: Retrospective analysis of medical records of neonates admitted to Cardiac Intensive Care Unit in whom diagnosis of left-sided obstructive heart defect was established. RESULTS: During 8-year period records of 117 patients were evaluated. Of those, 83 had hypoplastic left heart syndrome, 13 aortic stenosis, 12 coarctation of aorta, and 9 interruption of aortic arch. In 13 (11%) patients diagnosis of heart defect was established prenatally, other neonates presented postnatally with heart failure (28.2%), shock (65.4%) or severe cyanosis (4.8%). Continuous infusion of PGE1 and dopamine was administered in 84.6% and 30.8% patients, respectively. Thirty-two (30.8%) patients were mechanically ventilated and 36 (34.6%) received sodium bicarbonate. In referring hospitals the heart defect was most often diagnosed using echocardiography and diagnosis was established accurately in 55% patients. CONCLUSION: The study showed that in primary care hospitals neonates with known or suspected left-sided obstructive heart disease need more aggressive resuscitative measures than those used in this patient series. Prenatal diagnosis rate of these heart defects is very low (Tab. 3, Fig. 1, Ref 8). Full Text (Free, PDF) www.bmj.sk.

Immunologic status in pediatric cardiosurgical patients with chylothorax.

OBJECTIVE: The impact of lymphocyte and immunoglobulin loss on immunologic status has not been extensively studied in children with chylothorax. The purpose of this study was to evaluate immunologic profile of pediatric cardiosurgical patients who developed infection while suffering from prolonged postoperative chylothorax. METHODS: We retrospectively reviewed immunologic findings in 16 pediatric cardiac patients with post-operative chylothorax persisting ?7 days. Patients were on total parenteral nutrition, received colloides for replacement of chylous losses, and antibiotics and/or antimycotics for treatment of infection. Immunologic evaluation included immunoglobulin levels, cellular immunity, and phagocytic activity. For every parameter z-score was calculated according to age-dependent nomograms and t-test was used to compare z-score distribution with normal distribution. RESULTS: The immunoglobulin (IgG, IgM, and IgA) levels did not significantly differ from normal values, although 25% patients had IgG levels below normal range. The relative and absolute counts of peripheral blood lymphocytes were lower (p < 0.001) than normal values. Absolute numbers of blood B-lymphocytes (CD19+), T-lymphocytes (CD3+), helper/inducer T-cells (CD4+), and suppressor/cytotoxic T-cells (CD8+) were also below normal range (p < 0.001); however, their relative percentages and a CD4+/CD8+ ratio were within normal limits. The percentage and absolute number of natural killer cells (CD16+), phagocytic and metabolic activity of polymorphonuclear leukocytes did not differ from normal values. CONCLUSIONS: Persisting chylothorax results in B-cell and T-cell lymphopenia with proportional decline of CD4+ and CD8+ cells. Hypogammaglobulinemia observed in other studies has not been detected in this series probably due to administered plasma. Effects of these immunologic alterations on development of infection are unknown (Tab. 2, Ref. 13).

Sildenafil as a pulmonary vasodilator after repair of congenital heart disease.

We describe successful use of enteral sildenafil following surgery for congenital heart disease in three cases. One infant after repair of ventricular septal defect and aortic coarctation had pulmonary hypertension non-responsive to nitric oxide, another infant and 3.5 year child following palliative surgery for congenital heart disease with univentricular physiology were treated with inhaled nitric oxide and had severe systemic desaturations associated with endotracheal suctioning. Therapy with sildenafil reduced pulmonary arterial pressure, prevented episodes of arterial desaturations and allowed weaning from nitric oxide (Ref. 7). Full Text (Free, PDF) www.bmj.sk

Procalcitonin, neopterin and C-reactive protein after pediatric cardiac surgery with cardiopulmonary bypass.

BACKGROUND: Kinetics of activation of newly introduced inflammatory markers is of particular value in their use in postoperative setting after pediatric cardiac surgery. OBJECTIVE: To assess the influence of cardiopulmonary bypass (CPB) on activation of inflammatory markers. METHODS: Procalcitonin, neopterin and C-reactive protein (CRP) blood levels were measured before, on day 1 and day 2 after surgery in 152 pediatric patients undergoing cardiac surgery with CPB. All patients had infection-free postoperative course. RESULTS: Procalcitonin blood levels increased from 0.08 (0.001-0.19) ng/mL before surgery to 0.79 (0.13-3.5) ng/mL on day 1 (p < 0.001) and 0.52 (0.07-2.7) ng/mL on day 2 (p < 0.001) after surgery. Procalcitonin levels on day 1 correlated with CPB duration, cross-clamping time and use of aprotinin. Neopterin values increased from 8.4 (4.4-32) nmol/L before surgery to 16.1 (6.8-37.6) nmol/L on day 1 (p < 0.001) and 10.9 (5.3-31.1) nmol/L on day 2 (p < 0.001) after surgery. Neopterin levels on day 1 correlated negatively with age, lowest esophageal temperature and use of aprotinin; and positively with circulatory arrest, length of stay and use of modified ultrafiltration. CRP values increased from 4.7 (2.7-9.6) mg/L before surgery to 38.8 (13-73.5) mg/L (p < 0.001) on day 1 and 38.3 (15-88) mg/L (p < 0.001) on day 2 after surgery. CRP levels correlated positively with age at surgery, use of aprotinin and lowest esophageal temperature; and negatively with circulatory arrest and modified ultrafiltration. CONCLUSIONS: Procalcitonin and neopterin levels peaked on day 1, CRP levels remained elevated during first two postoperative days. Neopterin and CRP had age dependent kinetics (Tab. 2, Fig. 2, Ref. 18). Full Text (Free, PDF) www.bmj.sk.

Is a learning curve for arterial switch operation in small countries still acceptable? Model for cooperation in Europe.

OBJECTIVES: To assess the results of a cooperative arrangement between Slovakia and Slovenia for neonatal cardiac surgery. The aim of the study was to analyze the performance of this approach for complete transposition of the great arteries (D-TGA). METHODS: Due to the overall small number of new patients with D-TGA in Slovenia a decision was made to avoid a prolonged learning curve by centralizing the experience of two countries at one center. Since 1995 the center in Slovakia has become the only referral center for Slovenia. Between February 1993 and June 2002 in this center, 147 patients with D-TGA underwent arterial switch operation (ASO). The median age at operation was 11 days, with 110 patients from Slovakia and 37 patients from Slovenia. RESULTS: Overall hospital mortality was 4.8% (seven patients). The 1, 2, 3, 4 and 5 year survival rate was 95% with the mean follow-up of 4 years. Operation before 1997 (P=0.0001) was identified as a risk predictor for death by multivariate analysis. There are no deaths among the 90 patients operated on after 1996. All patients are without medication with normal left ventricular function. Stenosis (gradient >30 mmHg) was noted in the pulmonary artery reconstruction in seven patients (5%). More than mild aortic regurgitation was noted in five patients (4%). The incidence of redo or reintervention was 5% at 5 years of follow-up. CONCLUSIONS: In the current era a prolonged learning curve for ASO is not acceptable to most European countries and their patients. The risk of surgery can be minimized by concentrating surgical experience as part of the quality control of congenital heart programs. If the number of new patients is small due to the birth rate and size of the population, institutions should merge activity. Such centralization amplifies the experience to the benefit of the patient.

Thyroid hormone metabolism in pediatric cardiac patients treated by continuous povidone-iodine irrigation for deep sternal wound infection.

OBJECTIVE: The purpose of this study was to assess the influence of povidone-iodine mediastinal irrigation used for the treatment of deep sternal wound infection (DSWI) on thyroid function. METHODS: Thyroid function was studied in 18 pediatric cardiac patients treated with continuous povidone-iodine irrigation for DSWI. The median age of patients was 8 months (18 days-5.3 years). Serum concentrations of total triiodothyronine (TT3), total thyroxine (TT4), free triiodothyronine (FT3), free thyroxine (FT4), thyroid-stimulating hormone (TSH), reverse triiodothyronine (rT3) and thyroxine-binding globulin (TBG) were measured at three time points: (a) prior to mediastinal reexploration (before povidone-iodine exposure); (b) immediately after discontinuation of povidone-iodine irrigation; (c) 2 weeks after discontinuation of mediastinal irrigation. Urinary iodine excretion was examined on the last day of povidone-iodine exposure. RESULTS: Prior to the mediastinal reexploration, the median TT3 and TT4 levels were below the normal range, then increased significantly to concentrations within the normal range. The median serum FT3 levels were within the normal range throughout the observation period, though a significant increase of FT3 levels was observed after discontinuation of irrigation. The median serum FT4 concentrations were within the normal range prior to irrigation and did not change significantly. The median rT3 levels were within the normal range, close to upper normal limit. The median TBG levels were within the normal range throughout the observation period, though a significant increase of TBG levels was observed during the period of mediastinal irrigation. The median TSH level was within the normal range prior to mediastinal irrigation and did not change significantly. Urinary iodine concentrations in infants with povidone-iodine irrigation were significantly higher 6700 microg/l (range, 1600-15000 microg/l) than in the group of 53 healthy infants 200 microg/l (range, 20-780 microg/l, P<0,001). CONCLUSIONS: Our data showed that the use of povidone-iodine irrigation in the patients with DSWI has not lead to any significant alteration in thyroid function within the study period.

[High-frequency pressure ventilation with high positive end-expiratory pressure in the treatment of respiratory failure in neonates]. / Vysokofrekvencná tlakovo riadená ventilácia s vysokým pozitívnym koncovoexspiracným tlakom v liecbe respiracného zlyhania novorodencov.

The authors present results of group of 13 neonates treated with high frequency positive pressure ventilation (HFPPV) with high positive end-expiratory pressure (PEEP) for severe respiratory failure. The ventilatory protocol was based on the following principles: a) higher mean airway pressure (MAP) to achieve adequate oxygenation, b) MAP titrated mainly with PEEP, c) fraction of inspired oxygen (FiO2) below 0.6, d) small tidal volumes 3-6 ml/kg, e) ventilatory rates to achieve normocapnia in newborns with persistent pulmonary hypertension and to allow permissive hypercapnia in others. During HFPPV, the maximum values for respiratory rate, PEEP, MAP and peak inspiratory pressures (PIP), the incidence of airleak and the need for inotropic support were recorded. The values for arterial partial pressure of oxygen (paO2), FiO2, paO2/FiO2 and MAP during conventional ventilation and 30 minutes after initiation of HFPPV were statistically analyzed. paO2 increased from 8.0 kPa (3.3-10.4) to 11.8 kPa (7.3-16.2, p < 0.001) and paO2 (torr)/FiO2 increased from 62.2 (24.7-101.2) to 157.5 (62.2-275.2, p < 0.001) 30 minutes after institution of HFPPV when MAP was increased from 11.8 cmH2O (9-13.8,) to 17.2 H2O (14.8-22.2) p < 0.001. This allowed turning down FiO2 from 1 (0.6-1) to 0.6 (0.4-1 p < 0.001). Maximal ventilatory rates used were in average 60/min (50-105), PEEP 8 cmH2O (6-10), PIP 30 cmH2O (26-45), MAP 18.8 cmH2O (14.8-22.2). Air leak did not occur in any patient. Catecholamines were used in 8 patients. The duration of ventilatory support lasted in average 6 days (2-18). All patients were successfully extubated. 5 of them required nasal continuous positive airway pressure (14 hours--7 days). (Tab. 3, Fig. 3, Ref. 19.)

[Peritoneal dialysis in children after cardiac surgery]. / Peritoneálna dialýza u detí po kardiochirurgických operáciách.

The authors analyse their experience with the use of peritoneal dialysis (PD) in children with acute renal failure after cardiac surgery. From 1995 through 1998, 1246 children were operated in Pediatric Cardiocenter 38 of them developed acute renal failure and required peritoneal dialysis. Indications for PD were oliguria, anuria, hyperkalemia and fluid overload with generalized tissue oedema. PD was instituted at mean 33.9 (3-132) hours after surgery and lasted mean 53.7 (6-264) hours. None of the patients developed peritoneal infection. Hyperglycaemia developed in 8 patients and was treated with continuous insulin infusion. 5 patients required catheter reposition or reinsertion due to inadequate fluid removal and 1 patient required another renal replacement therapy. 22 (57.9%) patients died due to intractable low cardiac output. 16 survivors recovered their renal functions. In a long-term follow-up, 7 patients had no signs of significant renal dysfunction. In 4 patients, mild elevation of N-acetyl-beta-D-glukozaminidase indicates mild proximal tubular dysfunction. These findings together with aminoaciduria in 5 patients will require a further follow-up. (Tab. 2, Fig. 1, Ref. 16.)