Right ventricle to pulmonary artery conduit: a comparison of long-term graft-related events between bovine jugular vein conduit, aortic homograft, and porcine-valved conduits.

Background: The optimal conduit for right ventricle to pulmonary artery (RV-PA) reconstruction does not exist. Reinterventions are common due to conduit stenosis and endocarditis. Tailoring conduit choice according to patients' characteristics could improve the outcomes. The study aimed to compare graft-related events (infective endocarditis, transcatheter pulmonary valve replacement (PVR), transcatheter conduit dilatation, surgical conduit replacement, and transcatheter pulmonary branch intervention for RV-PA reconstruction using bovine jugular vein, aortic homograft, and porcine-valved conduits. Methods: In a retrospective cohort study, 155 patients with 193 procedures that were done in King Faisal Specialist Hospital and Research Center-Jeddah (KFSHRC JED) for implanting RV-PA conduits from 1999 to 2021 were included. The patients were grouped according to the type of conduit into 3 groups; Group 1 (n=153) received bovine jugular vein (BJVs) grafts, Group 2 (n=29) received aortic homografts, Group 3 (n=11) received porcine-valved conduits. Factors associated with graft-related events were evaluated using stepwise Cox regression analysis. Results: Patients with BJVs were significantly younger than those with porcine-valved conduits 3 (P=0.009). The weight of BJVs patients was significantly lower than homografts (P=0.002) and porcine-valved conduits patients 3 (P<0.001). The conduit size was as expected significantly lower in BJVs patients than patients who received porcine-valved conduits (P<0.001) and patients who received aortic homografts (P<0.001). There was no difference between Group 2 and 3 (P=0.084). Operative mortality occurred in 13 patients: 12 (7.84%) with BJV conduit and 1 (9.09%) with porcine valved conduit (P=0.351). Male gender [odds ratio (OR): 10.04; 95% confidence interval (CI): 1.28-78.86; P=0.028] and smaller conduit size (OR: 0.78; 95% CI: 0.61-0.99; P=0.048) were associated with increased operative mortality. Freedom from graft-related events at 5 and 10 years was 67% and 52% in BJVs patients, 74% and 36% in patients who received aortic homografts, and 53% in patients who received porcine-valved conduits. Factors associated with increased graft-related events were male gender (HR: 1.58; 95% CI: 1.004-2.50, P=0.048) and younger age (HR: 0.995; 95% CI: 0.991-0.999, P=0.041). Conclusions: RV-PA reconstruction was associated with low mortality, unrelated to the conduit type. Reinterventions for graft-related events were common. The durability and graft-related events might be comparable among BJV grafts, aortic homografts, and porcine-valved conduits. Factors associated with increased graft-related events in this study were male gender and younger age.

Effectiveness of alteplase infusion for the management of prosthetic mitral valve thrombosis in paediatric age group and proposed algorithm.

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.

A single-centre study of genetic mutations, audiology, echocardiogram and pulmonary function in Saudi children with osteogenesis imperfecta.

OBJECTIVES: Osteogenesis imperfecta (OI) is a heterogeneous group of inherited connective tissue disorders, characterised by skeletal fragility. Patients with OI may also exhibit extra-skeletal features like blue or grey scleral colour, fragile skin, easy bruising, joint laxity, short stature, deafness, cardiac valve abnormalities and abnormal pulmonary function. The objective of this study is to describe genetic mutations, prevalence of hearing issues, cardiac complications and impaired pulmonary function in children with OI. METHODS: This is a cross-sectional study of 23 Saudi children aged 6 months to 18 years who were diagnosed with OI. The revised Sillence classification (2,105) was used to classify the OI type. Whole exome sequencing was performed for genetic mutations. The hearing was assessed by either pure-tone audiometry and/or otoacoustic emission testing. Cardiac defects were screened by echocardiograms. Spirometry was performed to assess pulmonary function. Data were analysed with descriptive statistics. RESULTS: Based on the Sillence classification, 16 patients had OI type III, 6 had type IV and 1 had type I. Of the18 patients who had genetic sequencing, 66.6% had autosomal dominant and 33.3% had autosomal recessive mutations. Among children who had screening, hearing loss was diagnosed in 53% (9/17), congenital cardiac malformations in 26% (5/19) and restrictive lung disease in 70% (7/10). CONCLUSIONS: We found significant extra-skeletal features and a high yield of genetic mutations associated with OI. We suggest further studies to develop a screening protocol for extra-skeletal features in children with OI.

Safety and efficacy of transcatheter closure of atrial septal defect type II under transthoracic echocardiographic guidance: A case control study.

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 (<13 years old). For the other 10 patients, we could not find a matching pair. The data of the study group were compared with an age, weight, and height matched group (controls), who underwent the procedure under transesophageal echocardiography guidance. RESULTS: The diameter of the atrial septal defect, septal length, and most of the rims were comparable. The superior rim and inferior rims were longer in the study group. The devices chosen for the cases were larger than the control group. Procedure time and fluoroscopy times were shorter in the study group. Success rate was comparable. On follow-up, both groups had almost no or minimal incidence of residual shunt. CONCLUSION: We conclude that transcatheter closure of atrial septal defect under fluoroscopy and transthoracic echo guidance is safe and successful in selected patients who have single central atrial septal defect with adequate septal lengths and adequate septal rims, with high incidence of complete occlusion rate.

Rare combination of bilateral divided atrial chambers and pulmonary vein stenosis with review of the literature.

INTRODUCTION: The term cor triatriatum is used to describe a fibromuscular partition that divides an atrium into two compartments. It was first used by Borst in 1905, although the lesion had been described before by Church in 1868. Both described divided left atrial chamber (also referred to as cor triatriatum sinister). The incidence of the divided left atrial chamber among patients with congenital heart disease is reported at 0.1% and 0.4% in an autopsy study. Divided right atrial chamber - also referred to as cor triatriatum dexter - is even rarer to an extent that there is no reported incidence and only few hundred cases have been reported so far. A search through pubMed library revealed only 90 publications on humans, either under the term "dexter" or "dextrum". We would like to report a new association of bilateral divided atrial chambers and pulmonary vein stenosis, with a review of the literature.

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates.

The Saudi Association for Pulmonary Hypertension (previously called Saudi Advisory Group for Pulmonary Hypertension) has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008.[1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension (PH). One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension in children.

Pulmonary hypertension (PH) is relatively uncommon in children. Pulmonary arterial hypertension (PAH) in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. Most common PAH in pediatric are idiopathic (IPAH) or PAH associated with congenital heart disease (PAH-CHD), while other associated conditions, such as connective tissue disease (CTD), are less common in pediatrics. Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm.

Stent protrusion in palliative congenital heart disease interventions: does it cause any harm?

BACKGROUND: Protrusion of the patent ductus arteriosus (PDA) stent can occur into the lumen of the main pulmonary artery (MPA) branch, the aorta, or both. This protrusion can vary from trivial to major, causing potential obstruction to the vessel lumen, which may cause flow obstruction or risk of thromboses. As far as we know, no one has followed these patients with protruding stents to see whether they do pose a risk of obstruction or thromboses. METHODS: A retrospective, descriptive, cross-sectional study reviewing charts of all included patients who received stents in the MPA branches with residual protrusion into the pulmonary artery branch lumen (total, 87 patients; 34 patients with protruding stents) was performed to determine whether this protrusion caused any undesired effects on flow or coagulation. The patients were divided into two groups: the protruding stents group (group 1); and the non-protruding stent group that served as a control group (group 2). Each group was then categorized into 3 sections according to the stent position, the PDA, the MPA branches, and the Blalock-Taussig shunt. RESULTS: The only risk factor that had statistical significance was the number of stents in the PDA site. CONCLUSION: Protruding stents do not cause an increased risk of thrombosis in patients on aspirin. Mild protrusion is more likely in PDA stents and severe protrusion is more likely in the MPA branch stents. Severe protrusion is more likely when more stents are used in the PDA location. There is no statistical evidence that protrusion can cause lung perfusion defects from the small numbers we have.

Right superior vena cava draining in the left atrium associated with tetralogy of Fallot and pulmonary atresia.

We report a case of an anomalous drainage of the right superior vena cava to the left atrium with intact atrial septum associated with Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries.

Venovenous malformation: a common finding after Kawashima operation.

OBJECTIVES: It has been reported that systemic venovenous malformation (VVM) can develop in patients with interrupted inferior vena cava (IVC) and univentricular type of congenital heart disease who undergo superior vena cava to pulmonary artery connection (Kawashima operation). These malformations can lead to profound systemic desaturation postoperatively. However, there have been few reports that characterise the prevalence, anatomic details and clinical correlations of these systemic VVM arising after Kawashima operation. In this study, we describe our experience with VVM after Kawashima operation, and discuss issues regarding their evaluation and postoperative management. METHODS: Eight patients with median age 19 months (range: 5-238) who underwent Kawashima operation were subjected to postoperative angiography, prospectively. Sites of VVM origin and entry, as well as their course, were documented. The presence of pulmonary arteriovenous malformations (AVMs) was also documented. RESULTS: At median follow-up of 31 months (range: 16-72 months), a total of 14 VVM were found in different supra- and infra-diaphragmatic sites in six patients (75%); four of them had concomitant pulmonary AVM while the remaining two patients had only pulmonary AVM. CONCLUSIONS: Our findings suggest that systemic VVM can occur frequently after Kawashima operation and can produce significant desaturation postoperatively, and hence we support hepatic incorporation. Performing detailed angiographic studies of the supra- and infra-diaphragmatic systemic veins in routine assessment of patients before Kawashima operation is, probably, warranted.

Late presenters with dextro-transposition of great arteries and intact ventricular septum: to train or not to train the left ventricle for arterial switch operation?

OBJECTIVE: We report our experience in managing late presenters (older than 4 weeks) with dextro-transposition of great arteries and intact ventricular septum (d-TGA/IVS) in an effort to achieve successful arterial switch operation (ASO) in a third world setting. DESIGN: We retrospectively reviewed the charts of all late presenters with d-TGA/IVS. Patients were divided into two groups: left ventricular training (LVT) group and non-left ventricular training (non-LVT) group. LVT group underwent pulmonary artery banding and Blalock-Taussig Shunt prior to ASO. RESULTS: Twenty-one late presenters were included in the study. In LVT group, 11 patients with median age of 6 months (range, 1-72 months) underwent LVT. Later, 8 patients with median age of 9.25 months (range, 1.33-84 months) underwent ASO. Prior to ASO, left ventricle (LV) collapse resolved in all and left ventricle to systemic pressure (LV/SP) ratio was 0.81 (range, 0.76-0.95) in 4 patients. Two patients who had LVT for < or =14 days required postoperative extracorporeal membrane oxygenation (ECMO) support due to LV dysfunction. Seven patients survived to discharge. In non-LVT group, 10 patients with median age of 2.5 months (range, 1-98 months) underwent ASO. Five patients had LV collapse, and median LV/SP ratio was 0.67 (range, 0.56-1.19) in 5 patients. Seven patients needed ECMO support. Seven patients survived to discharge. CONCLUSION: Late presenters with d-TGA/IVS, who have LV collapse on echocardiography and/or a LV/SP ratio <0.67 on cardiac catheterization, should be subjected to LVT preferably for duration of longer than 14 days in order to avoid potential ECMO use.

Hemolytic anemia: an unusual presentation of cor triatriatum sinistrum.

Cor triatriatum is a congenital malformation of the heart which is usually presented with heart failure. We have dealt with an unusual presentation of a two-month-old child with cor triatriatum presented with hemolytic anemia.

Propranolol: a new indication for an old drug in preventing postoperative junctional ectopic tachycardia after surgical repair of tetralogy of Fallot.

Junctional ectopic tachycardia (JET) is a major cause of postoperative morbidity after complete repair of tetralogy of Fallot (TOF). Propranolol is a known medication used in patients with TOF to prevent and control hypercyanotic spells. Despite this, there is little information regarding the relation between preoperative use of propranolol and the incidence of postoperative JET. The aim of this study was to examine the effect of preoperative use of propranolol on the incidence of postoperative JET after full surgical repair of TOF. A retrospective analysis of 109 patients in whom 57 patients received preoperative propranolol (propranolol group) was compared with 52 patients who did not receive propranolol preoperatively (control group). The incidence of postoperative JET was significantly higher in the control group (38%) than the propranolol group (21%) P=0.042. The propranolol group had significantly less mechanical ventilation time, less ICU stay and less total hospital stay than the control group (P<0.05). Our findings suggest that the preoperative use of propranolol may decrease the incidence of JET after full surgical repair of TOF. A prospective randomized study may help to elucidate the exact relationship between the preoperative use of propranolol and the incidence of postoperative JET.

Alpha(2)-blocker helps to avoid systemic to pulmonary shunt in a prostaglandin dependent infant with critical pulmonary valve stenosis.

A 27 days old newborn with critical pulmonary valve stenosis remained prostaglandin (PGE(1)) dependent for 2 weeks after successful balloon valvuloplasty. Only the introduction of Phentolamine in his medication regimen, allowed PGE(1) to be weaned off within days of this therapy. The medication was continued for 4 days and replaced by angiotensin converting enzyme inhibitor (Captopril). Few weeks after the discharge, the patient remained clinically stable with acceptable saturation.

Acute myocardial ischemia following accidental intravenous administration of epinephrine in high concentration.

Inadvertent and accidental epinephrine overdose might result in potentially lethal complications. We present a case of acute epinephrine toxicity resulting in acute myocardial ischemia in a young boy with combined variable immunodeficiency syndrome who developed severe allergic reaction to intravenous immunoglobulin, and was subsequently given epinephrine by mistake intravenously rather than subcutaneously. He developed significant ischemic changes in standard 12-lead electrocardiogram, transiently raised cardiac enzymes, reduced left ventricular systolic function, pulmonary edema and pulmonary hemorrhage. It is suggested that special precautionary measures should be taken regarding the dose and the route while administering epinephrine to avoid mishaps.

Resternotomy for a retrosternal cardiac pseudoaneurysm in a 1.5-year- old child: a case report.

Cardiac pseudoaneurysm is a contained rupture of the myocardium that can occur after cardiac surgery, chest trauma, and endocarditis. The wall of the pseudoaneurysm consists of fibrous tissue and lacks the structural elements found in a normal cardiac wall, and it is contained by the pericardial adhesions or the epicardial wall. Early surgery is recommended even for asymptomatic patients due to the propensity for rupture and fatal outcome. We report our experience with the surgical approach of a child with a cardiac pseudoaneurysm who had undergone a biventricular repair of a double outlet right ventricle with non-committed ventricular septal defect in the form of intraventricular tunneling.

Stent implantation to maintain patency of a stenosed Blalock Taussig shunt.

A 14-year-old female with complex congenital heart disease underwent a left-sided classical Blalock Taussig (BT) shunt 15 days after birth. Ten years after the operation her oxygen saturation had decreased significantly. An angiography revealed a severely stenosed BT shunt. Balloon dilation including implantation of a 6 x 13 mm stent was performed successfully. Immediately after intervention, oxygen saturation rose from 55% to 80 84% in room air. Follow-up at a year and a half later showed the classical BT shunt was still patent.

The efficacy and safety of the Amplatzer ductal occluder in young children and infants.

BACKGROUND: We have used the Amplatzer ductal occluder for transcatheter closure of large persistently patent arterial ducts, and used our experience to assess the safety and efficacy of the device in young children and infants. METHODS AND PATIENTS: We used the Amplatzer ductal occluder prospectively in 43 patients with large patent arterial ducts, reviewing our experience to identify any problems or complications. RESULTS: The procedure proved successful in 42 of the patients. We achieved complete occlusion of the duct in 33 (78.5 per cent) of the patients on the day of insertion. In 6 additional patients, complete occlusion occurred 1 week to 6 months after the procedure. Trivial leaks persisted in 2 patients, while one had a significant residual leak. Problems were encountered in 7 patients. The procedure failed in one, a device was wasted in 2, pulled through in 3, while we experienced kinking of the long Mullins sheath, being unable to retrieve the device, in one patient. Minor complications occurred in 6 patients, finding flow at a peak velocity of 2.2 metres per second in the descending aorta in 2 patients, and at 2.5 metres per second in 2 further patients, and flow at 2.5 metres per second in the pulmonary arteries of two patients. One patient experienced a major complication due to excessive bleeding. Out of the 14 patients suffering adverse events, 13 weighed less than 10 kilograms. This rate of problems and complication in these patients weighing less than 10 kilograms was significantly higher than in the patients weighing more than 10 kilograms. CONCLUSION: Transcatheter occlusion of moderate to large patent arterial ducts with the Amplatzer ductal occluder device is safe and effective, with a high rate of complete occlusion. Problems and minor complications may be encountered in children weighing less than 10 kilograms. If the device is to be deployed completely in the ductal ampulla, and to avoid descending aortic obstruction, the size of the retention flanges of the occluder should not exceed the largest diameter of the patent arterial duct.

Left ventricular dysfunction after closure of large patent ductus arteriosus.

Changes in left ventricular dimensions and performance were studied in 43 patients after transcatheter occlusion or surgical ligation of patent ductus arteriosus. The patients were assigned to 2 groups based on their ductal diameter: >/= 3.1 mm to group A (n = 27) and