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.

Double aortic arch anomalies in Children: A Systematic 20-Year Single Center Study.

Aortic arch anomalies underlie numerous congenital disorders. Effectively diagnosing and treating them requires close understanding of cardiovascular embryology. As our Center serves the entire pediatric population of our country, we performed a comprehensive retrospective analysis of all aortic arch anomalies diagnosed at our Center over the past 20 years. We analyzed 40 children with aortic arch anomalies, distinguishing two defect types: Group 1 displayed ring-forming anomalies, and Group 2 other types of aortic arch anomalies that did not form a vascular ring. We performed detailed morphological analyses using echocardiography, angiography, computed tomography, or magnetic resonance imaging and generated a catalog of all aortic arch anomalies present. Group 1 was represented by 25 patients; 40% with persistent both aortic arches, and 60% with various forms of right aortic arch and an incomplete left aortic arch. Group 2 was represented by 15 patients with complex heart defects. On the basis of our dataset, the incidence of all aortic arch anomalies was 0.033%, and of ring-forming pathologies 0.021%. Although aortic arch anomalies are rare, it is important to diagnose them correctly. It is critical to distinguish ring-forming types. Although in complex defects the aortic arch anomaly represents only an additive diagnosis, its correct definition could be crucial for further management. Cumulatively, this unique, long-term study provides a systematic patient registry and offers critical epidemiological data to aid the study of rare congenital cardiovascular defects. Clin. Anat. 30:929-939, 2017. © 2017 Wiley Periodicals, Inc.

Stenting of stenotic aortopulmonary collaterals in non-surgically treated adolescent or adult patients with pulmonary atresia, ventricular septal defect and multiple aortopulmonary collaterals.

OBJECTIVES: To present our experience with stent implantations in non-surgically treated patients with pulmonary atresia, ventricular septal defect and stenotic aortopulmonary collaterals. METHODS: Between April 2007 and June 2009, 6 stents in 4 patients were implanted into stenosed segments of MAPCAs. The median age and weight of patients at stent implantation were 24 years (range 13-34 years) and 58.5 kg (range 56-70 kg), respectively. RESULTS: All implantations were successfully performed; there were no procedure associated complications. The median diameter increase of stenosed MAPCAs was 107%. The mean arterial blood saturation increased from mean 78% to 84%. An early increase in the median exercise duration measured by 6MWT was 36%. Neointimal in-stent fibroproliferation in 4/6 stents and stent fracture in 1/6 patients occured in mid-term follow-up. CONCLUSION: Percutaneous catheterizational stent implantation into stenosed segments of MAPCAs is a palliative procedure, that might improve quality of life of non-surgically treated patients with PA, VSD, MAPCAs. Neointimal in-stent fibroproliferation and stent fracture can be expected as complications in mid-term follow-up. CT-angiography is recommended to confirm these complications in progressive satO2 decrease (Fig. 3, Ref. 12).

Experiences with fenestration closure in patients after the Fontan operation.

BACKGROUND AND OBJECTIVES: [corrected] In this study, decision and timing of fenestration closure are discussed. We review our experiences with transcatheter occlusion of fenestration or residual baffle leak using Amplatzer occluder. PATIENTS AND METHODS: We have retrospectively reviewed the cath lab records of 26 patients who have undergone closure of fenestration (or another baffle leak) in period of 2002 to July 2006. Patients with the previous fenestrated Fontan procedure (intracardiac lateral tunnel, extracardiac conduit) with the presence of clinically important cyanosis, arterial desaturation less than 88 %, previously described leak by echocardiography, impaired exercise capability, absence of effusions and appropriate hemodynamic parameters were included in the study. RESULTS: The most frequent group were patients with hypoplastic left heart syndrome (HLHS) (27 %), second most common was L-transposition of great vessels (TGA), tricuspidal valve atresia (TA) and atresia of pulmonary artery (PA) (all of them 15.4 %). CONCLUSIONS: Our results confirm that after adjusting the hemodynamical risk, transcatheter fenestration occlusion in high risk patients might be a safe and effective method to significantly increase arterial O2 saturation. The long term benefits of closure especially at exertion are yet to be shown (Tab. 2, Ref 21). Full Text (Free, PDF) www.bmj.sk.

Echocardiographic measurements of the aorta in normal children and young adults.

BACKGROUND: To be able to determine aortic valve and ascending aorta pathology, especially aortic root dilatation, it is important to establish normal aortic dimensions. The aim of the study was to measure the dimensions of the aorta in normal healthy children and young adults in Slovakia. PATIENTS AND METHODS: 702 healthy subjects, from newborns to 20 years of age, were examined at our institution. The study was carried out prospectively, by a single observer, using digitized two-dimensional (2D), Doppler and M-mode echocardiography. The aorta was measured at 3 sites: 1. aortic valve annulus, 2. sinuses of Valsalva, 3. sinotubular junction. Patients were divided into 28 groups according to their body surface area (BSA)--from 0.15 to 2.0 m2. All data were statistically evaluated (mean value, 5th and 95th percentile for all BSA groups) and regression equations were calculated for each parameter. RESULTS: All 3 measured aortic parameters correlated closely. Measures of correlation (R-squared) for aortic parameters with the square root of BSA were high: 0.89 for aortic valve annulus, 0.86 for sinuses of Valsalva and 0.86 for sinotubular junction (Tab. 3, Fig. 7, Ref 13). Full Text (Free, PDF) www.bmj.sk

[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.)

Abnormal electroretinography in schizophrenic patients with a history of sun gazing.

Electroretinographic (ERG) measurements were performed in 9 schizophrenic patients and in 13 control subjects. The measurements of schizophrenic patients as a group did not differ from those of normals. However, 6 schizophrenic patients who had a past history of sun gazing showed a decrease in retinal responsiveness under conditions of light adaptation. These results suggest that a subgroup of schizophrenic patients, who show deviant light-related behavior, have abnormal ERG. We postulate that an abnormality in retinal dopaminergic neurons, which are known to reduce light responsiveness of horizontal and ganglion cells, is the underlying pathophysiology of this clinical finding.