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Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
The Rastelli procedure is used to treat D-transposition of the great vessels with a ventricular septal defect and pulmonary stenosis. The ventricular septal defect is closed so that the left ventricle empties into the aortic valve. The native stenotic pulmonary valve is excised and closed, and a conduit is placed from the right ventricle to the pulmonary arteries to provide pulmonary blood flow.
Congenitally corrected transposition of the great arteries
Published in Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček, Congenital Heart Disease in Adults, 2008
Jana Popelová, Erwin Oechslin, Harald Kaemmerer, Martin G St John Sutton, Pavel Žáček
This procedure can be performed if pulmonic stenosis and a large VSD are present. The procedure consists of switching the venous return at atrial level (Senning’s or Mustard’s operation) and performing a Rastelli procedure. During the Rastelli procedure, the morphologic left ventricle is directed to the aorta by a tunnel between the VSD and the aortic valve. A conduit is placed from the morphologic right ventricle to the bifurcation of the pulmonary artery. By this procedure, the morphologically left ventricle becomes the systemic ventricle.
Management of congenitally corrected transposition from fetal diagnosis to adulthood
Published in Expert Review of Cardiovascular Therapy, 2023
All the surgical options mentioned above leave the morphologically RV in the systemic position. A completely new approach to surgical management of ccTGA was proposed at the turn of the 1980s and 1990s [34–36]. The aim of so-called anatomical correction or double-switch procedure is to restore the morphologically left ventricle into the subaortic position. It can be achieved by atrial switch (Mustard/Senning procedure) in combination with arterial switch operation. In patients with pulmonary valve stenosis, the arterial switch procedure cannot be performed. In such cases, the Rastelli procedure, the Nikaidoh procedure and the REV procedure are the options [37]. The long-term survival of anatomic repair is acceptable (20-year survival of 83%) [38], and the long-term complication of the arterial switch is the neoaortic regurgitation [39]. Of importance, this surgical option is available only for infants and young children in whom the morphologically left ventricle is still capable to sustain systemic pressure. In patients without subpulmonary ventricular flow obstruction, the pulmonary artery banding has been proposed as a way to adapt the left ventricle to work as a systemic ventricle. The results of pulmonary artery banding as a bridge to double switch procedure are inconsistent [40–42]. An intention-to-treat analysis of pulmonary artery banding showed that patients above 16 years of age are unlikely to achieve anatomic repair [43]. Some authors noted that individuals with palliative pulmonary artery band had better survival than the anatomic repair group [44], whereas others observed the lowest transplant-free survival at 10 years in this group of patients [45].
Updates in the management of congenital heart disease in adult patients
Published in Expert Review of Cardiovascular Therapy, 2022
Danielle Massarella, Rafael Alonso-Gonzalez
Ascertaining incidence of SCD in patients with CHD is challenging. Luckily, SCD in patients with CHD is relatively rare at the general population level, with a yearly incidence rate between 0.07 and 0.40 per 100,000 persons-year [26]. However, among CHD cohorts, the risk has been estimated between 0.28% and 2.7% per year [25,29–32], which is 20–30-fold higher than the general population [26]. Due to the heterogeneity and small relative size of the patient population identifying the patients with highest risk is challenging. Long-term follow-up studies suggest that SCD accounts for up to 26% of late mortality following surgical intervention for congenital heart disease [33]. Outcome data in the current surgical era suggest that this has decreased to 7–13% [34,35]. Several studies have identified certain subpopulations that could be at higher risk. Gallego et al. identified d-TGA patients repaired with atrial switch as the population with highest risk of SCD (10 per 1,000 persons-year) in a cohort of 936 patients with CHD over 9 years of follow up [31]. Oliver et al., in a more contemporary cohort that merged retrospective data with a case-control study, identified 278 cases of SCD (n = 163) or non-fatal cardiac arrest (n = 115). CHD lesions were classified as high, moderate, and low risk. Patients with the higher risk (incidence rate of SCD >12%) were patients with Rastelli procedure, severe coronary anomalies, complex tetralogy of Fallot and cyanotic patients. Patients with non-complex tetralogy of Fallot, atrial switch for d-TGA, ccTGA, Fontan circulation and Ebstein anomaly, were at moderate risk (incidence rate 4–12%), whereas patients with coarctation of the aorta were at low risk (incidence rate <1%-4%) [36].
Cefpodoxime proxetil as a therapeutic option in switching therapy for infective endocarditis in children: case reports and literature review
Published in Journal of Chemotherapy, 2019
Nina Krajcar, Lorna Stemberger Marić, Dalibor Šarić, Neven Milić, Goran Tešović
A 6.5-year-old male child was admitted to the UHID on the 26th day of febrile illness. The patient had a history of congenital heart disease and underwent surgical conduit replacement after total correction of the truncus arteriosus communis (type I according to Collett and Edwards) by Rastelli procedure 5 years prior to current illness. On the 1st day of acute onset of a high-grade fever up to 40 °C, a 14-day course of oral amoxicillin/clavulanate therapy was started. Other symptoms included headache, sore throat, abdominal pain and vomiting. After three days of therapy the fever resolved, but on the 12th day of disease, fever increased up to 39 °C accompanied with night sweats and chills. The treatment was switched to oral cefuroxime axetil but without any clinical effect. On the 21st day of illness, the patient was hospitalized in a local hospital in Coastal Croatia with presumptive diagnosis of infective endocarditis and empiric intravenous therapy with vancomycin and meropenem was initiated. The patient became afebrile the following day but diagnostic tests didn′t confirm the diagnosis of endocarditis therefore he was transferred to the UHID. On admission, the patient was well-appearing, afebrile and with normal vital signs. Physical examination revealed a pansystolic murmur best heard over the 3rd and the 4th left intercostal spaces. The patient’s WBC count was 7000/microL and his C-reactive protein level was 20.6 mg/L. In the next 3 days, the antibiotic therapy was discontinued and several blood cultures were obtained which were all negative. On the 4th day of hospitalization, the patient became febrile again, up to 39.1 °C.The laboratory findings showed elevated C-reactive protein (234.3 mg/L) and erythrocyte sedimentation rate (85 mm/hour). Furthermore, mild anaemia (RBC 3.78 × 106/microL; haemoglobin 9.4 g/dL; hematocrit 28.6%) and thrombocytopenia (127 000/microL) were registered. After blood cultures were collected, the treatment with vancomycin (45 mg/kg/day divided q8h) and ceftriaxone (80 mg/kg/day in one daily dose) was initiated. The patient`s clinical condition improved rapidly and he became afebrile within 48 hours. Despite the fact that TTE as well as heart MR imaging didn`t reveal vegetations, infective endocarditis could not have been eliminated due to dysplastic aortic valve and degenerated conduit. PCR analysis of blood, using primers targeting the 16S rDNA sequence, was negative as well as the results of serologic examinations for Q fever and Brucella. However, one blood culture taken during febrile episode came positive for Haemophilus parainfluenzae. Since the organism was sensitive to third-generation cephalosporins, 9 days after the initiation vancomycin was discontinued and ceftriaxone monotherapy was administered for the next 6 days. Intravenous treatment was followed by oral cefpodoxime proxetil (10 mg/kg/day divided q12 h) for 6 weeks in total. Blood cultures drawn during and after IV therapy remained negative and the patient was discharged with full recovery. In the following two years after hospital discharge, the patient was in good condition, afebrile, without complaints and relapses of IE.