Non-invasive assessment of haemodynamic parameters in patients after Fontan procedure.

INTRODUCTION AND OBJECTIVE: Single ventricle anomaly is one of the complex congenital heart defects. A dependable non-invasive method of evaluation of Fontan circulation haemodynamics for early diagnosing unstable patients is hardly available in routine clinical practice. The aim of the study is non-invasive evaluation of the haemodynamic parameters in patients after Fontan operation. MATERIAL AND METHODS: The study involved 11 participants (age 24.4±4.3 years) with functionally univentricular hearts after Fontan operation. Evaluation of haemodynamic parameters was performed in supine and sitting positions using the impedance cardiography method. RESULTS: In comparative analysis, heart rate (70.1 vs.78.3 1/min; p=0.001), diastolic blood pressure (73.9 vs. 76.7 mm Hg; p=0.026), mean arterial blood pressure (84.5 vs. 88.0 mm Hg; p=0.013), systemic vascular resistance (1284.8 vs. 1334.9 dyn*s*cm-5; p=0.024), systemic vascular resistance index (2178.7 vs. 2272.8 dyn*s*cm-5*m2 ; p=0.018), pre-ejection period (124.2 vs. 136.2 ms; p=0.009), systolic time ratio (0.43 vs. 0.53; p=0.0001), and Zo (26.2 vs. 28.7 Ω; p<0.00001), were significantly higher in the sitting position. Stroke volume (75.4 vs. 68.5 ml; p=0.013), stroke index (42.7 vs. 39.0 ml*m-2; p=0.014), thoracic fluid content (38.5 vs. 35.4 1*kΩ-1; p=<0.00001), thoracic fluid content index (22.8 vs. 21.0 1*kΩ-1*m-2; p=<0.00001), and leftventricular ejection time 291.1 vs. 260.1 ms; p <0.00001, were significantly higher in the supine position. CONCLUSIONS: In patients after Fontan procedure, impedance cardiography can be a useful tool the assessment of shortterm haemodynamic changes provoked by postural changes. Its clinical value in patients with congenital heart defects should be further investigated.

Children and Adolescents with Pulmonary Arterial Hypertension: Baseline and Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

We present the results from the pediatric arm of the Polish Registry of Pulmonary Hypertension. We prospectively enrolled all pulmonary arterial hypertension (PAH) patients, between the ages of 3 months and 18 years, who had been under the care of each PAH center in Poland between 1 March 2018 and 30 September 2018. The mean prevalence of PAH was 11.6 per million, and the estimated incidence rate was 2.4 per million/year, but it was geographically heterogeneous. Among 80 enrolled children (females, n = 40; 50%), 54 (67.5%) had PAH associated with congenital heart disease (CHD-PAH), 25 (31.25%) had idiopathic PAH (IPAH), and 1 (1.25%) had portopulmonary PAH. At the time of enrolment, 31% of the patients had significant impairment of physical capacity (WHO-FC III). The most frequent comorbidities included shortage of growth (n = 20; 25%), mental retardation (n = 32; 40%), hypothyroidism (n = 19; 23.8%) and Down syndrome (n = 24; 30%). The majority of children were treated with PAH-specific medications, but only half of them with double combination therapy, which improved after changing the reimbursement policy. The underrepresentation of PAH classes other than IPAH and CHD-PAH, and the geographically heterogeneous distribution of PAH prevalence, indicate the need for building awareness of PAH among pediatricians, while a frequent coexistence of PAH with other comorbidities calls for a multidisciplinary approach to the management of PAH children.

A rare cause of newborn central cyanosis.

Pulmonary arteriovenous malformations are rare congenital vascular anomalies. They are usually associated with congenital hemorrhagic hemangioma. The hemodynamic effect of fistulas depends on their size, as well as the location. The most common manifestations include central cyanosis, ischemic stroke, murmur over the lung fields, cardiomegaly and, less often, heart failure. We present the case of a child who was admitted to the Department of Pediatric Cardiology and Congenital Heart Defects due to central cyanosis and heart murmur.

Cardiac tumors in children: A 20-year review of clinical presentation, diagnostics and treatment.

BACKGROUND: The use of new imaging techniques has contributed significantly to earlier diagnosis and treatment of cardiac tumors. OBJECTIVES: The aim of the study was to analyze data from children with cardiac tumors in terms of clinical presentation, the role of noninvasive diagnostic procedures and the long-term outcome. MATERIAL AND METHODS: The data analyzed retrospectively concerned 30 children in whom cardiac tumors were diagnosed from January 1995 to July 2015. The cardiac evaluation included a review of the subjects' medical records and medical history, a physical examination, standard 12-lead electrocardiography, echocardiography and 24-h Holter ECG monitoring at the time of diagnosis and at 6-12 month intervals during the follow-up at the authors' outpatient clinic. RESULTS: Most of the children did not need cardiac surgery; surgical tumor excision was necessary in 3 cases only. There was 1 death in the follow-up period. Rhabdomyoma was diagnosed in 22 cases, and in 16 of them tuberous sclerosis was confirmed during the follow-up period. In the remaining 8 cases, fibroma was the most likely diagnosis. CONCLUSIONS: The symptomatology of cardiac tumors in children can vary greatly, from the absence of any symptoms up to heart failure and respiratory distress indicating the need for surgical intervention. The diagnosis of cardiac tumors relies almost exclusively on noninvasive imaging techniques. The observations in this study confirm the fact that the most common cardiac tumor in children is rhabdomyoma, which may disappear spontaneously. Most patients with cardiac tumors do not require treatment.

Unusual location of the Libman-Sacks endocarditis in a teenager: a case report.

Libman-Sacks endocarditis may be the first manifestation of systemic lupus erythematosus. The risk of its occurrence increases with the co-existence of the anti-phospholipid syndrome. Changes usually involve the mitral valve and the aortic valve. In this report, we present a case of Libman-Sacks endocarditis of the tricuspid valve in a teenage girl.