Browsing by Author "Lubaua, Ingūna"
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Item Ebstein anomaly and right aortic arch in patient with Charge syndrome(2021-11-13) Lubaua, Ingūna; Teraudkalna, Madara; Department of PaediatricsEbstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle. It often coexists with other congenital cardiac malformations. Up to 79–89% of patients with Ebstein anomaly have interatrial communication in the form of patent oval foramen or atrial septal defect and more than one-third has other types of cardiac malformations. Association between Ebstein anomaly and right aortic arch is extremely rare and only few cases have been described in the literature so far. Much rarer than with other cardiac malformations, Ebstein anomaly is associated with non-cardiac malformations or genetic syndromes. Several cases of association between Ebstein anomaly and Charge syndrome have been reported, nevertheless, Ebstein anomaly accounts for less than 1% of cardiac defects seen in patients with Charge syndrome. In this case report, we present a unique case of a patient with Charge syndrome where both Ebstein anomaly and right aortic arch are present. The diagnosis of Ebstein anomaly and right aortic arch was established prenatally. In the first years of life, the patient did not exhibit any remarkable symptoms. However, over time, deterioration of right ventricle function and increased tricuspid regurgitation were observed, requiring consideration of surgical treatment at the age of five. In addition, delay in physical, motor, and mental development was observed and thus, at the age of five, the patient was consulted by a medical geneticist and a gene panel to test for structural heart defects was ordered. The test showed a mutation in chromodomain helicase DNA binding protein 7 (CHD7) gene, which, along with clinical features, allowed to establish a diagnosis of Charge syndrome. To the best of the authors’ knowledge, this is the first case report of a patient with Charge syndrome, Ebstein anomaly, and right aortic arch that has been described in the literature.Item Hypoplastic left heart syndrome : a review(2016) Gobergs, Roberts; Salputra, Elza; Lubaua, Ingūna; Rīga Stradiņš UniversityBACKGROUND: Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of the disease led to death, usually within the first weeks of life. Recently surgical palliative techniques have been developed allowing for a five-year survival in more than half the cases. MATERIALS AND METHODS: We reviewed literature available on HLHS, specifically its anatomy, embryology and pathophysiology, and treatment. The Pubmed and ClinicalKey databases were searched using the key words hypoplastic left heart syndrome, foetal aortic valvuloplasty, foetal septoplasty, Norwood procedure, bidirectional Glenn procedure, Fontan procedure, hybrid procedure. The relevant literature was reviewed and included in the article. We reported a case from Children's Clinical University Hospital, Riga, to illustrate treatment tactics in Latvia. RESULTS: There are three possible directions for therapy in newborns with HLHS: orthotopic heart transplantation, staged surgical palliation and palliative non-surgical treatment or comfort care. Another treatment mode - foetal therapy - has arisen. Staged palliation and full Fontan circulation is a temporary solution, however, the only means for survival until heart transplantation. Fifty to 70% of patients who have gone through all three stages of palliation live to the age of five years. CONCLUSIONS: The superior mode of treatment is not yet clear and the management must be based on each individual case, the experience of each clinic, as well as the financial aspects and will of the patient's parents.Item ISOLATED COR TRIATRIATUM SINISTER : A CASE REPORT(2020) Grīnberga, Zanda; Sīlis, Pauls; Ligere, Elina; Lubaua, Ingūna; Bergmane, Inta; Auziņa, Luīze; Smits, Lauris; Ozolins, Valts; Sikora, Normunds; Lāce, Inga; Rīga Stradiņš UniversityCor triatriatum sinister is a rare congenital cardiac anomaly that has been identified in 0.1% of children with congenital heart disease. It is defined as a fibromuscular membrane that divides the left atrium into two chambers: a superior (proximal) that in most cases receives drainage from the pulmonary veins and an inferior (distal) chamber that communicates with the mitral valve and the left atrium. Cor triatriatum sinister can be an isolated lesion (approximately 25% of cases), but in many cases it is associated with other congenital cardiovascular anomalies, the most common one being – atrial septal defect(3). Symptoms in patients with cor triatriatum sinister are related to obstruction of pulmonary venous drainage, pressure loading of the right side of the heart and congestive cardiac failure. Depending on the severity of the obstruction and presence of associated cardiac anomalies it can be diagnosed at any age. Diagnosis is usually achieved by echocardiography in early infancy. Elective treatment method is surgical excision of the membrane. Here we present a pediatric patient (4 months old) presenting in cardiogenic shock with a successful correction of isolated cor triatriatum sinister. To confirm diagnosis and success of surgical repair, transthoracic and transesophageal echocardiography were used.Item Multisystem Inflammatory Syndrome Cardiovascular Complications in Patient associated with SARS-CoV-2 Infection(2023-01-20) Kaķe, Marta; Šmitiņš, Emīls; Apine, Ilze; Lubaua, Ingūna; Department of Paediatrics; Department of RadiologyCases of long-term outcomes of a novel disease MIS-C have rarely been reported. The most common cardiac manifestations of MIS-C are myocarditis, coronary artery aneurysms, conduction abnormalities, and arrhythmias. We report a case of an 8-year-old boy, who has Asperger syndrome, with a complicated course of disease called MIS-C during Covid-19 outbreak, when this was the first diagnosis of MIS-C in Latvia. The patient was tested positive for the SARS-CoV-2 (RNA) and Hemophilus influenza (DNA). In 9th day of hospitalization, the diagnosis of MIS-C was performed. He had involvement of gastrointestinal, cardiovascular, respiratory and coagulation systems. The patient received therapy with IVIG, Anakinra as well antibiotics and cardiovascular medicine. During hospitalisation patient had complications such as anemia of a combined nature, post-infectious bone marrow suppression, hepatosplenomegaly and candidiasis due to CVC. The cardiac magnetic resonance imaging (cMRI) after being diagnosed with MIS-C revealed edema and focal changes in LV inferior wall, although volume of both ventricles and LV systolic function were normal. A year after the follow-up cMRI, showed resolution of the myocardial edema. In existing literature there are several cohort studies about long-term outcome of MIS-C myocarditis in 6 month follow-up visit, showing no evidence of scar tissues in cMRI, but in some patients- diastolic dysfunction was detected. This report emphasizes a complicated form of disease in patient who has Asperger syndrome with excellent outcome.Item MULTISYSTEM INFLAMMATORY SYNDROME IMPACT ON THE CARDIOVASCULAR SYSTEM : SINGLE-CENTRE STUDY OF LATVIA(2024-02-01) Šmitiņš, Emīls; Gardovska, Dace; Lubaua, Ingūna; Rīga Stradiņš UniversityMIS-C (Multisystem inflammatory syndrome in children) is a hyperinflammatory syndrome caused by the Sars-CoV-2 virus, still an ongoing issue worldwide. MIS-C is associated with an impairment of various organ systems, including the cardiovascular system, and up to 100% of all MIS-C patients have a broad spectrum and severity of symptoms. Identifying MIS-C early and starting therapy is crucial to minimise possible complications and clinical worsening. A prospective cohort study in a single centre was conducted at the Children’s Clinical University Hospital in Latvia from January to December 2021. Patients between the ages of one and seventeen years who met the MIS-C criteria were included in the study. We evaluated the patient's demographic data, blood pressure, echocardiographic data, ESG data, and cardiac biomarkers such as proBNP and troponin I. Thirty-one patients were included who met the MIS-C criteria. The median age was 8.0 years, and 52% were boys. Of all patients, 77% initially presented with hypotension, and 42% required inotropic support. Treatment in the paediatric intensive care unit (PICU) was required in 58% of patients. Reduced left ventricular ejection fraction was observed in 35% of patients. Mildly decreased ventricular ejection fraction (< 55%) was observed in 19% of cases, and moderate dysfunction (ejection fraction < 45%) in 16% of patients. Twelve per cent of patients received milrinone to improve left heart function. Left heart function significantly improved in all patients during the hospitalisation. In 6% of all patients, coronary artery dilations were observed. All patients had dilation resolution at the time of discharge. The median length of hospitalisation was twelve days, and the median length of PICU stay was three days. Multisystem inflammatory syndrome in children is a significant and potentially life-threatening illness with cardiovascular involvement in 100% of cases. Patients who present primarily with higher ProBNP levels are more likely to have decreased left ventricle ejection fraction, which should be kept in mind when evaluating patients with MIS-C. Overall, patients with MIS-C have a good prognosis, and most cardiovascular changes have been resolved by discharge, but further follow-up and studies are needed to judge the long-term outcome.Item Prevalence of patent ductus arteriosus in children under 18 years in Latvia 2018‑2022(2023) Paņina, Aleksandra; Ozolins, Valts; Smits, Lauris; Sikora, Normunds; Ligere, Elīna; Lubaua, Ingūna; Bergmane, Inta; Lāce, Inga; Sīlis, Pauls; Rīga Stradiņš UniversityIntroduction: Accounting for 5‑10% of all congenital heart defects, patent ductus arteriosus (PDA) is one of the most common congenital cardiovascular malformations. The condition is often diagnosed in the neonatal period. Howev‑ er, it can be delayed until childhood or even adulthood. A small PDA often doesn’t cause problems and may never need treatment, but a large, untreated PDA can reduce heart function or problems related to high pressure in the lungs. Aim: Analysis and summary of data from medical history of patients with PDA in the system of the Children’s Clinical University Hospital. Materials and Methods: The study included 70 patients diagnosed with PDA aged 0‑18 years from term infants. Data were analyzed using the diagnostic criteria for PDA. Results: A total of 70 patients were enrolled in the study, of whom 71% (n=50) were female and 29% (n=20) were male. Most patients (60%) were children aged 0 to 3 years (n=42). The small and moderate PDA type predominate, each type is 44% (n=31) by PDA criterion. When analyzing one of the most important echocardiographic criteria for PDA, the LA /AO ratio, pulmonary hyperperfusion was present in 53% (n=36) of patients. Angiography to close the PDA was performed in 94% (n=66) of cases, open surgery was performed in 3% (n=2) of cases, and surgery was discontinued in 3% (n=2) because of disease. Of the total number of patients, complications occurred in only 5,8 % (n=4), including migration of the occluding device 3% (n=2), residual PDA 1,4% (n=1), and a small piece of the encapsulating ligature 1,4% (n=1). Conclusions: By 2021, 359 000 children had been registered in Latvia, and ~ 0,018 % of children aged 0‑18 with PDA. For children born full‑term, early diagnosis and correction of PDA enables a good long‑term quality of life.