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Introduction:The incidence of prosthetic valve implantation is increasing in the paediatric population. Prosthetic valve thrombosis leading to obstruction could potentially be a life-threatening complication. There is a debate regarding optimal management of this complication, and there is limited use of thrombolytic therapy in childhood in the setting of valve thrombosis.Objective:We aim to share our experience of successfully using fibrinolytic therapy in terms of alteplase for paediatric prosthetic mitral valve thrombosis and to propose a management algorithm.Methods:This retrospective analysis of the database was conducted at our hospital including patients who underwent thrombolysis (alteplase) for prosthetic mitral valve thrombosis from June, 2011 to June, 2021. A total of 10 patients with 20 attempts of alteplase infusion were found in our record.Results:Alteplase was successful in 19 attempts to relieve valve thrombosis. The safe and effective dose of alteplase was between 0.1 and 0.3 mg/kg/hour. There were no associated major bleeding complications and alteplase was administered either by central or peripheral line.Conclusion:Thrombolysis by alteplase infusion was found to be successful in relief of prosthetic mitral valve thrombosis in paediatric population without major bleeding complications.

Aims: To understand the accuracy of non-invasively obtained blood pressure gradients (cuff vs Doppler gradient) with an invasively measured pressure gradient. Study Design: Retrospective study. Place and Duration of Study: Department of Pediatrics, Section of Pediatric Cardiology, King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia, between Jan, 2010 till Jan, 2020. Methodology: A retrospective study of patients with CoA who underwent cardiac catheterization between Jan, 2010 till Jan, 2020 was performed. Cuff BP gradients and Doppler echocardiography were measured prior to cardiac catheterization and afterwards, when an intervention was performed. Student t test and Bland-Altman analysis were performed. Results: 55 patients with aortic coarctation underwent 92 cardiac catheterizations. Among them 75 needed interventions. This resulted in a total of 162 cardiac catheterizations (cath), from them 67 pressure gradients included in the analysis. There was no statistically significant difference between mean Doppler measurements and invasively derived catheter gradients (p=0.12). In contrast peak Doppler measurement (p < 0.00001) and cuff blood pressure gradients (p=0.03) showed significant differences to the cath gradient. We found that cuff blood pressure gradients accurately reflected cath measurement in native COA (p=0.40) and in those who weigh less than 10 kg (p=0.67). Mean Doppler measurements had a small tendency for underestimation. Peak Doppler gradient and cuff pressure gradient usually overestimated cath measurement. Conclusion: The mean Doppler echocardiography seems to be the most accurate among the other noninvasive methods in use to estimate severity of aortic coarctation. It provided reasonable agreement with the invasively obtained aortic coarctation gradient.

Background Assessment of the shape and dimensions of PDA is usually done angiographically and in the majority of cases need arterial access. Our aim was to evaluate the value of routine 2 D echocardiography (ECHO) in predicting type, dimensions of PDA and to anticipate device size to be used during the intervention. Material and methods The charts of all patients who underwent transcatheter closure of PDA between January 2015 and December 2020 were reviewed. Their pre-procedure ECHO and catheterization details at the time of device closure were analyzed. Results Total of 139 patients were reviewed and 8 were excluded because of lack of adequate echocardiographic or angiographic images. The mean age and weight of the study population were 2.6 ± 2.5 years (range 0.2–14 years) and 11.2 ± 7.8 kg (range: 1.5–57 kg), respectively. There was no statistically significant difference in PDA narrowest diameter (p = 0.99) and predicted device type (p = 0.54) between Echo and angiography. Echo slightly overestimated PDA length (p = 0.01) and aortic ampulla dimension (p = 0.047), while morphology of PDA was correctly identified in the majority of cases (82%). Conclusions Pre-procedure echocardiography correlates well with angiographically obtained measurements and hence can be used to estimate PDA diameter, shape and guide device decide selection. Routine echocardiography can be used successfully to plan the intervention and in some cases to guide transcatheter closure.

Background: Transcatheter closure of secundum atrial septal defect is routinely performed under general anesthesia and transesophageal echocardiography guidance. If patients have good echo windows, the procedure could be performed under transthoracic echo guidance. Aim of study: To evaluate safety and efficacy of the intervention using fluoroscopy and echo guidance. Methods: In a case control study design, 180 patients underwent atrial septal defect closure between January 2010 and December 2016. In 32 patients, the intervention was performed under fluoroscopy and transthoracic echo guidance. Our study group consisted of 22 out of 32 patients (

Background Dilated cardiomyopathy (DCM) is characterized by left ventricular dilation and dysfunction. The association with significant mitral regurgitation worsens the prognosis. Case report A 2-year-old girl presented with DCM and severe mitral regurgitation. She had a history of viral myocarditis at the age of 4 months, necessitating recurrent hospital admissions for management of intractable heart failure, pneumonia, and failure to thrive. The decision was taken to proceed for mitral valve surgery, which ended with mitral valve replacement. Over 3 years of follow-up after surgery, there was significant improvement in her weight gain and she improved clinically. There were still recurrent admissions, but mostly for adjustment of her deranged anticoagulation medications. Conclusion Mitral valve surgery might be indicated in selected patients with DCM.

Management of neonatal native coarctation is debated till now. Surgical therapy remains an option but may be unwarranted in critically sick infants with complex lesions. Balloon dilatation has been employed but with early re-stenosis. Stent angioplasty has also been used but as a bridge towards definitive surgical therapy. Four critically sick infants with complex coarctation and additional co-morbidity factors underwent primary stent therapy as surgical intervention was denied. One patient had died earlier due to reasons unrelated to the procedure. Three survivors underwent multiple dilatations of primary stents as indicated. One of the three survivors did not require any further dilatation after the age of 5 years and remained stable till the time of reporting. High-pressure Cheatham Platinum stents were implanted inside the primary stents in two infants, who developed re-stenosis due to somatic growth. These stents were further balloon dilated at high atmospheric pressure. Femoral arteries in both of them were blocked but were re-canalized after balloon dilatation in one and stent angioplasty in the other. After a follow-up of about 15 years, all of them have been doing fine with acceptable Doppler gradients. They were normotensive and on no cardiac medications. It can be concluded that, though surgical repair remains a standard of care, stent angioplasty in selected infants with complex lesions is feasible and effective. Multiple dilatations can be performed without added risk of stent migration. Bio-absorbable and growth stents hold a promise for future use in such situations.

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E'- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance.

Background Recanalization of arterial duct (AD) is rarely needed. Objective The aim of this study is to report our experience regarding the feasibility and effectiveness of arterial duct recanalization in three infants and review the relevant literature. Methods and results We report on three patients with decreased pulmonary blood flow after initial palliation. The first patient had pulmonary atresia (PA) and an intact ventricular septum. He needed recanalization of the AD after pulmonary valve perforation and dilatation. The second patient had PA and ventricular septal defect (VSD). His AD originated from the left subclavian artery. He required AD recanalization after palliation with a central shunt and clipping of the duct. During intervention he developed a thrombus in the stent, which was treated successfully using thrombolytic treatment. The third patient had PA and VSD. The arterial duct originated from the left subclavian artery and was recanalized after spontaneous closure despite prostaglandin infusion. During the procedure the patient had severe desaturation and bradycardia requiring resuscitation for two minutes. All infants had successful arterial duct recanalization and stenting. Additionally, they were clinically stable during follow-up. Conclusion Arterial duct recanalization and stenting is a feasible and effective procedure in selected cases, and its risks are treatable. Long-term studies are required. (Less)

Percutaneous closure of patent ductus arteriosus (PDA) is done in the cardiac catheterization laboratory, usually under fluoroscopic and angiographic guidance. The aortogram is used for assessing PDAsize and shape the pre-device implantation. Additionally, post-device aortograms are applied for the assessment of device position, profile and residual shunt. Angiograms expose patient to radiation and possible untoward effects of contrast media. Recently, transthoracic echocardiography has been utilized to guide in PDAclosure to avoid radiation exposure as well as contrast material. On two occasions, we were obligated by special circumstances to close the PDAunder echocardiography guidance. First case was a 6-month baby girl with mild signs of heart failure having moderate size PDA. She underwent device closure under transthoracic echocardiogram as the angiographic system had stopped working during the procedure. Second case was a 6-year girl, weighting 16-kg with chronic renal failure. She had moderate size PDAclosed by device under transthoracic echocardiographic, guidance avoiding the use of contrast agent due to chronic renal failure.

Life-threatening intracardiac and great vessels thrombi are rare in neonates. Recombinant tissue plasminogen activator (rTPA) is used in adults to stimulate fibrinolysis and facilitate thrombus resolution. Its use in neonates, along with heparin, remains controversial because of potential risk of serious bleeding. We aim to present our experience with the use of thrombolytic agents in seven neonates and young infants. In a retrospective study, over a period of 6 years, the medical records of neonates and young infants, who were diagnosed with intracardiac and great vessels thrombi, were reviewed. The following factors were collected: demographic data, primary diagnosis, thrombus site, risk factors, method of diagnosis, thrombolytic and/or anticoagulation agent, route, dose and duration of treatment, complications, and outcome. Six neonates and one 45-day-old infant were analyzed. Age ranged from 5 to 45 days (median age 12 days), and median weight was 2.9 kg (range 0.9-3.8 kg). The thrombi were diagnosed by echocardiography in five and by angiography in two cases. All patients had life-threatening thrombi; four were treated with rTPA (0.5 mg kg(-1) h(-1)) and heparin infusions with complete dissolution of the thrombi, within a median time of 60 h (6-72 h), and without complications. The remaining three patients (two who were premature, at 28 and 34 weeks of gestation, and the third who had a deranged coagulation profile) were treated with unfractionated heparin due to fear of bleeding. The thrombi dissolved in the premature babies (within 2 weeks and 3 months, respectively) but embolized and resulted in the death of the third infant after 2 weeks of treatment. The current case series confirmed the effectiveness and safety of intravenous rTPA infusion, at the dosages used, in neonates and young infants with life-threatening thrombi.

Background Coarctation of aorta may present as severe heart failure in infants and may lead to myocardial dysfunction. Current evidence supports surgical management of neonatal coarctation. However, it can be precarious in critically sick infants. Aim and objective To investigate the safety, efficacy, immediate and mid-term outcome of percutaneous balloon angioplasty (BAP) for infantile aortic coarctation in critically sick patients. Methods and patients Data of all patients under age of 6 months who underwent balloon angioplasty for coarctation were reviewed. Results Between January 2008 and April 2014, 15 infants were identified with coarctation with a mean weight of 3.4 kg (1.4–5 kg) and median age of 54 days (4–142 days). All patients were critically sick, needed admission in intensive care unit for inotropic or ventilatory support. All underwent successful percutaneous balloon angioplasty with no major complications. The clinical condition and left ventricular function improved leading to discontinuation of prostaglandin and weaning from inotrope and ventilator support. Seven patients underwent elective surgical repair after a mean time of one month, three needed re-dilation and five continued without any further intervention. Conclusions Surgical repair for native neonatal and infantile coarctation is a preferred choice of treatment but it can be challenging in critically sick patients. However, balloon dilation remains a safe and effective temporary palliation for the critically sick patients. Despite of high incidence of restenosis, some patients do not need for mid-term further intervention.

Congenital coronary artery fistulas are rare anomalies. Most of these fistulas drain to the right side of the heart. They seldom connect to either left atrium or left ventricle. We report a three years old girl in whom an atrial septal defect (ASD) was diagnosed. Echocardiography suggested a possible additional coronary artery fistula to the left ventricle (LV). Before closing the ASD, coronary angiography identified multiple coronary artery fistulas to the left ventricle. Transcatheter closure of the ASD was done successfully. The hemodynamic effect of coronary artery fistula to LV after ASD closure is discussed along with literature review of this extremely rare association.

An 18-year-old pregnant woman with severe pulmonary valve stenosis and exertional dyspnea underwent balloon dilation during pregnancy using sole echocardiographic guidance to protect the baby from radiation. The main technical difficulty encountered was during advancement of the catheter across the right ventricular outflow tract into the pulmonary valve. This was overcome using a wedge balloon catheter over a percutaneous transluminal coronary angioplasty (PTCA) wire. Using echo guidance, the balloon was positioned across the pulmonary valve and inflated. Pulmonary balloon valvuloplasty can be performed safely using sole transthoracic echocardiography guidance without fluoroscopy.