Assessment of 4D flow MRI for quantification of left-to-right shunt in pediatric patients with ventricular septal defect: comparison with right heart catheterization.

Objectives: The percentage of shunt fraction significantly impacts the management of patients with congenital shunts, influencing strategic choices such as surgical or interventional procedures. This study compared the estimated shunt fraction (the ratio of pulmonary-to-systemic flow, Qp/Qs) for quantifying the left-to-right shunt in children with ventricular septal defect (VSD) using heart catheterization, four-dimensional (4D) flow, and two-dimensional (2D) flow magnetic resonance imaging (MRI). The goal was to establish a non-invasive and reliable measurement ratio between pulmonary and systemic blood flow in these patients. Methods: Between July 2022 and June 2023, patients scheduled to undergo invasive right heart catheterization were included in this study. MRI was performed one hour before the catheterization procedure. The correlation of shunt fraction was assessed between all methods after calculating the Qp/Qs ratio from 2D and 4D flow MRI and catheterization. Results: A total of 24 patients (aged 3-15 years, eight females) were ultimately included in the study. The Qp/Qs ratios obtained from 4D flow had a robust correlation (correlation coefficient r = 0.962) compared to those obtained during catheterization. Cardiac catheterization recorded the mean shunt fraction at 1.499 ± 0.396, while 4D flow measured it at 1.403 ± 0.344, with no significant difference between the two techniques. Moreover, there was a reasonable correlation (r = 0.894) between 2D flow measurements of Qp/Qs and the results obtained from catheterization, with a mean shunt fraction of 1.326 ± 0.283. Conclusion: 4D flow MRI has the potential to be a non-invasive method for accurately measuring the left-to-right shunt in children with VSD.

Five-Year Outcomes of Coarctoplasty with Stents in the Pediatric Population: Results from a Retrospective Single-Center Cohort with Centrally Adjudicated Outcomes.

Transcatheter stent implantation is a widely performed procedure for treating native coarctation of the aorta (CoA) in pediatric patients. However, data on mid- to long-term outcomes are limited. The aim of this study was to evaluate the mid-term safety and efficacy of transcatheter CoA stenting based on centrally adjudicated outcomes. This retrospective cohort study included patients aged 15 years or younger undergoing de novo stenting for CoA or recoarctation (reCoA) between 2006 and 2017. Immediate and 5-year outcomes were assessed. Immediate outcomes (procedural and in-hospital) were retrieved from electronic records. Rates of 5-year reCoA, stent fractures, aneurysmal/pseudoaneurysmal formation, and all-cause mortality were mid-term outcomes. The study included 274 patients (64% male and 36% female) with a median (interquartile range) age of 9 (6-12) years. Procedural success was achieved in 251 patients (91.6%). Procedural complications occurred in 4 patients (1.4%), consisting of stent migration in 1 (0.3%) and small non-expanding non-flow-limiting aortic wall injuries in 3 (1.1%). Major vascular access complications were observed in 18 patients (6.6%), acute limb ischemia in 8 (2.9%). In-hospital mortality occurred in 4 patients (1.4%). Five-year cumulative incidence rates of stent fractures, reCoA, and aortic aneurysmal/pseudoaneurysmal formation were 17/100 (17%), 73/154 (48%), and 8/101 (7.92%), respectively. Of 73 reCoAs, 47 were treated with balloon angioplasty, and 15 underwent a second stent implantation. Five-year all-cause mortality occurred in 4/251 (1.6%) patients. Coarctoplasty with stents was safe and effective in our pediatric population during a 5-year follow-up despite a high rate of reCoA.

Ventricular Septal Defect and Mitral Regurgitation Due to Penetrating Cardiac Trauma; a Case Report and Review of Literature.

Penetrating cardiac trauma is a fatal condition and can result in the injury of various parts of the heart. Ventricular Septal Defect (VSD) following these traumas occurs only in 1-5% of cases. The patients' conditions depend on location, size, and concomitant injuries. One of the uncommon coincidences with the VSD is Mitral Regurgitation (MR) due to injury to sub-valvular structures. In this study, we report a case of concomitant traumatic-induced VSD and MR in a 14-year-old boy following a stab wound to his chest. The patient was a teenage boy coming to the Rajaei Cardiology Hospital emergency room following a stab wound to the anterior and left part of his chest. Despite primary urgent surgery, his breathlessness had continued for three more months. Evaluations with Transthoracic Echocardiography (TTE) revealed VSD with concomitant MR, but there was no papillary muscle rupture. Cardiac Magnetic Resonance Imaging (MRI) and angiographic evaluation confirmed the provisional diagnosis. The Amplatzer VSD occluder repaired the VSD, and the patient was discharged following the resolution of his symptoms. Although the MR has been present in the follow-up echocardiography, the patient has been asymptomatic. Since the initial presenting symptoms and signs of VSD and MR might be subtle or delayed, imaging modalities such as TTE and Transesophageal Echocardiogram (TEE) are beneficial in determining the diagnosis and the optimal treatment.

Long-term outcome of interventional approaches for treatment of coronary artery fistulas: a retrospective cohort study in a great referral center.

BACKGROUND: Despite the spontaneous regression of many cases of coronary artery fistulas (CAFs), symptomatic patients or cases with severe shunting may require therapeutic interventions. In the present study, we aimed to assess the outcome of treatment of CAFs using interventional approaches. METHODS: This retrospective cohort study was performed on 29 patients with CAFs that were referred to our tertiary center between 2009 and 2019. Baseline characteristics were collected by reviewing the hospital files, and the patients were followed up to assess long-term outcomes with a mean follow-up of 3.3 years. RESULTS: Overall, in 29 patients in our cohort study, 82.9% suffered from isolated CAFs and in the remaining cases, concurrent congenital abnormalities did exist. For treatment, coils (Cook, Pfm, Ev3) were used in 79.3%, ADO II(AGA) in 18.3%, vascular plug (AGA) in 3.4%, and a combination of coil/ vascular plug/amplatzer in 3.4%. Postoperative complications were reported in 4 patients as external iliac artery thrombosis, transient PSVT, ST-T wave changes and mild pericardial effusion that were all managed successfully with no adverse sequels. No coronary artery injury, device dislocation, dissection, ischemia or coronary dilatation occurred, and there was no death. As larger fistulas were treated by a retrograde approach through the right side of the heart, there was significant correlation between residual shunts and the mode of closure approach; the majority of the residual shunts occurred in patients in the retrograde approach group. CONCLUSIONS: Trans-catheter approach for treating CAFs leads to appropriate long-term outcome with minimal potential side effects.

Safety of Aortic Coarctation Treatment in Patients with Turner Syndrome: A Single-Country Case Series and Literature Review.

BACKGROUND: Coarctation of the aorta is a common cardiac disease in Turner syndrome. Evidence indicates that surgery and balloon angioplasty in infants and small children do not have any added risk of mortality or complication in these patients. Stenting in older patients may, however, pose higher risks of arterial wall injury and mortality. METHODS: In this case series, we describe 15 patients with coarctation of the aorta in Turner syndrome: 9 received stenting, 4 underwent surgery, and 2 were treated via balloon angioplasty. RESULTS: Dissection occurred in 2 patients after stenting: 1 in the aorta and the other in the external femoral artery. Both were managed promptly without any mortality or serious damage, one percutaneously and the other surgically. CONCLUSIONS: Awareness of increased risks and preparedness for prompt interventions in case of an acute arterial wall injury are recommended when coarctation stenting is done for a patient with Turner syndrome.

Current Endovascular Approach in Adult Patients with Pulmonary Vein Stenosis: A State-of-the-Art Approach.

Regarding to more invasive treatment of atrial arrhythmia (atrial fibrillation ablation) and pulmonary vein isolation (PVI), the rate of acquired pulmonary vein stenosis (PVS) is increasing and at present, PV ablation for AF has become the principal cause of PVS in adult patients. On the other way, by improvement in procedural techniques, equipment, and the experience of the operators, the incidence of PVS has been decreased. There is some controversy about the manner of follow-up of these patients and in most centers, just symptomatic patients are considered for imaging and treatment. Almost always, those with PV stenosis more than 70% or multiple PV involvement become symptomatic and if give them up without treatment, pulmonary symptoms and finally irreversible pulmonary hypertension will occurred. So, intensive pursue after the procedure is highly recommended. Whereas in pediatric patients with congenital or acquired PVS, the best treatment approach is surgery, in adult patients, the preferred type of treatment is the transcatheter intervention with high acute success rate. In this present review, we have scrutinized about the diagnostic modalities, the indications for intervention, the diverse treatment strategies, and principally clarify an accurate stepwise approach during transcatheter procedure.

Percutaneous Balloon Pulmonary Valvuloplasty of Critical Pulmonary Stenosis and severe pulmonary stenosis in Neonates and Early Infancy: A Challenge in the Cyanotic.

Introduction: Pulmonary stenosis with an intact ventricular septum (PS-IVS) is one of the common causes of cyanotic heart disease in neonates with diverse morphologies as well as management and treatment protocols. The aim of this study was to evaluate short and midterm results of balloon pulmonary valvuloplasty (BPV) for this disorder. Methods: Between 2012 and 2016, Totally 45 neonates and infants under 6 months old were evaluated.The patients had a minimum right-to-left ventricular pressure ratio of 1, right-to-left shunting at the patent foramen ovale or atrial septal defect level, and tricuspid valve Z-scores higher than -4. Results: Immediately after the procedure, the right ventricular pressure dropped to the normal values in 8 (20%) patients. The immediate procedural success rate was seen in 42 (93.3%) cases: the right-to-left ventricular pressure ratio dropped to below 50% or the level of O2 saturation rose above 75%. Of three cases unresponsive to BPV, two of them underwent patent ductus arteriosus (PDA) stenting and one procedural death occurred. At 6 months' follow-up, of 42 patients, this pressure was still with in the normal range in 36 (80%) infants, while it had returned to high values in 9 (20%) patients and necessitated repeat valvuloplasty. After BPV, severe pulmonary valve regurgitation was observed in14.2% patients; the condition was more common when high-profile noncompliant balloons were used. Conclusion: Balloon pulmonary valvuloplasty in infants with PS-IVS confers acceptable results insofar as it improves echocardiographic parameters and hemodynamic changes at short- and midterm followups.Balloon selection with sizes more than 1.2 of the diameter of the pulmonary valve annulus and the use of noncompliant high-pressure balloons results in higher degrees of pulmonary regurgitation.

Intravascular Ultrasound for Assessment of Residual Coarctation of the Aorta after Balloon Angioplasty in Infants.

Intravascular ultrasound (IVUS) has been introduced as an accurate and minimally invasive diagnostic technique for the assessment of vascular anatomy and its abnormalities. We believe that IVUS can be used for clarifying the reasons for failure of balloon angiography in infantile coarctation of the aorta (CoA), because post-balloon angioplasty tearing, intimal flap, thrombosis and pseudoaneurysm of the aorta can be evaluated by IVUS with greater sensitivity and specificity. We aimed to assess the outcome of balloon angioplasty of CoA using angiography as the gold standard and IVUS as a new method in infants, comparing the two techniques for the evaluation of the diameter and area of CoA segment pre- and post-procedure. This cross-sectional study was performed on 18 infants hospitalized with a final diagnosis of CoA. All the infants underwent angiography and were also assessed by IVUS to measure the preoperative and postoperative diameter of the narrow segment in the two anterior-posterior and lateral views. In assessment by IVUS, the mean diameter of the coarctation site increased from 2.10 ± 0.30 mm to 4.50 ± 0.94 mm (P < 0.001). Similarly, the average minimum area of the coarctation level increased from 5.26 ± 1.50 mm2 to 13.77 ± 3.48 mm2 after angioplasty (P < 0.001). Comparing these findings, angiography and IVUS showed a high level of agreement. In the assessment of a dissection flap, there was a high level of agreement between angioplasty and IVUS before the procedure, but IVUS had higher accuracy after the procedure. Our study showed that IVUS was more reliable than angiography in the assessment of residual coarctation. IVUS yielded high sensitivity (58.3%) and specificity (100%) for discriminating the presence and absence of residual coarctation as well as the need for repeating the procedure. The assessment of coarctation before and after angioplasty procedures in children is possible using the IVUS method, with high accuracy. IVUS can offer greater accuracy than angiography in the evaluation of the coarctation area, detecting tears, dissection and flaps, and assessment of residual coarctation.

Aspergillus flavus endocarditis and meningitis in a child with marfan syndrome.

BACKGROUND AND PURPOSE: Aspergillus species are implicated as the etiology of approximately 26% of endocarditis cases. Central nervous system aspergillosis is a life-threatening condition that has a mortality rate of 80%. CASE REPORT: Herein, we report a four- year- old female who was admitted to the pediatric infectious ward due to a fever of unknown origin in January 2020. She was a known case of Marfan syndrome with a family history of this syndrome in her mother. The species was identified using (PCR) and the antifungal susceptibility test was performed using four antifungal agents based on the Clinical and Laboratory Standards Institute M38 3rd edition. Fluconazole-resistant Aspergillus flavus was identified to be responsible for endocarditis and meningitis as well as fever of unknown origin. CONCLUSION: The clinicians should be aware and consider fungal endocarditis in blood culture-negative endocarditis even in patients with no significant risk factor when antibiotic therapy fails.

Left Atrial Drainage of the Right Superior Vena Cava: A Case Report.

An isolated right superior vena cava (RSVC) draining into the left atrium represents a very rare congenital malformation, especially in the absence of a partial anomalous pulmonary venous return. This condition leads to hypoxemia, cyanosis, and clubbing without any other signs of heart defects. We describe an 8-year-old girl, who was referred to our hospital due to unexplained cyanosis. Segmental approach in transthoracic echocardiography showed left atrial drainage of the RSVC, which was subsequently confirmed by contrast echocardiography and angiography. Surgical repair via trans-section and anastomosis of the superior vena cava to the right atrium was performed to prevent the complications of right-to-left shunting and cyanosis. During a 4-year follow-up, the patient remained in very good clinical status and her serial echocardiography was normal except for very mild left atrial and left ventricular enlargement.

Intraoperative diagnosis of a quadricuspid aortic valve.

Quadricuspid aortic valve is a rare anomaly, and most patients require surgery for aortic regurgitation in the 5th or 6th decades of life; only a few cases of aortic valve repair in childhood have been reported. A 3-year-old boy was scheduled for ventricular septal defect closure and aortic valve repair. Quadricuspid aortic valve was an incidental finding at operation; it was repaired by joining the left anterior and right anterior cusps. At the 9-month follow-up, the patient had no more than mild aortic regurgitation. We emphasize the importance of detecting this anomaly, especially in children with aortic valve regurgitation.

Pitfalls of stenting coarctation of an angulated right circumflex aortic arch in Goldenhar syndrome.

We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.

Predictors of Prolonged Mechanical Ventilation in Pediatric Patients After Cardiac Surgery for Congenital Heart Disease.

BACKGROUND: The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers. OBJECTIVES: The current study aimed to describe PMV predictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran. PATIENTS AND METHODS: From May to December 2014, all pediatric patients (less than a month - 15 years old) admitted to pediatric Intensive Care Unit (PICU) after congenital heart surgeries were consecutively included. The PMV was defined as mechanical ventilation duration more than 72 hours as medium PMV and more than seven days as extended PMV. The demographic data and variables probably related to PMV were recorded during the PICU stay. RESULTS: A total of 300 patients, 56.7% male, were enrolled in this study. Their mean age was 32 ± 40 months .The median duration (IQR) of MV was 18 hours (8.6 - 48 hours). The incidence of PMV more than 72 hours and seven days was 20% and 10.7%, respectively. Younger age, lower weight, heart failure, higher doses of inotropes, pulmonary hypertension, respiratory infections and delayed sternal closure were independent predictors of PMV in multivariate analyses. CONCLUSIONS: The results of this study indicated that PMV predictors could be specific for each center and a good administration program is needed for each pediatric cardiac surgery center for the preoperative management of patients undergoing congenital heart surgeries.

Left pulmonary artery stenting with glenn shunt: introducing a hybrid procedure.

Complexity of some congenital heart diseases sometimes necessitates a combination of interventional procedures and surgery, amongst which intraoperative stent implantation is one of the most common. We herein report a successful hybrid procedure in a cyanotic adult patient who had undergone no procedure in childhood. The patient was a 24-year-old cyanotic male (oxygen saturation in the room air was 65%) who presented with dyspnea. According to echocardiography, catheterization, and cardiac magnetic resonance imaging data, the patient was amenable to the Fontan surgery. However, because of significant left pulmonary stenosis and his age, he first underwent a hybrid procedure (Glenn shunt and left pulmonary artery [LPA] stenting). After the procedure, oxygen saturation rose to 83%. At six months' follow-up of the patient, exercise capacity and cyanosis had improved significantly, with O2 saturation having reached near 85% by pulse oximetry.

Percutaneous implantation of the Edwards SAPIEN(™) pulmonic valve: initial results in the first 22 patients.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) was introduced in 2000 as an interventional procedure for the treatment of right ventricular outflow tract (RVOT) dysfunction. The new Edwards SAPIEN(™) pulmonic valve has reached CE certification at the end of 2010 thus offering an attractive alternative with extended sizes (23 and 26 mm) to the conventional Melody(®) valve (sizes 18, 20 and 22 mm). PATIENTS: Over a 1-year period, PPVI using the Edwards SAPIEN(™) pulmonic valve was performed in 22 patients using a standardized procedure. Primary diagnosis was tetralogy of Fallot (n = 11), pulmonary atresia (n = 2), Truncus arteriosus (n = 3), TGA/PS-Rastelli (n = 1), Ross surgery (n = 2), double outlet right ventricle (n = 2) and absent pulmonary valve syndrome (n = 1). The character of the RVOT for PPVI was transannular patch (n = 4), bioprosthesis (n = 2), homograft (n = 5) and Contegra(®) conduit (n = 11). The leading hemodynamic problem consisted of a pulmonary stenosis (PS) (n = 2), pulmonary regurgitation (PR) (n = 11) and a combined PS/PR lesion (n = 9). RESULTS: In 21/22 patients, PPVI was performed successfully (10 × 23 and 11 × 26 mm). There were 9 female and 13 male patients; the mean age was 21.7 years (range 6-83 years), the mean length was 162 cm (range 111-181 cm) and the weight 56.5 kg (range 20-91 kg). Invasive data showed a decrease of RV-systolic pressure from 61.2 mmHg (± 23.1) to 41.2 mmHg (± 8.6) and reduction of RV-PA gradient from 37.3 mmHg (± 23.2) to 6.9 mmHg (± 5.3). The PA-systolic pressure increased from 25.8 mmHg (± 8.6) to 33.9 mmHg (± 9.3) as did the PA diastolic pressure (from 6.0 mmHg (± 5.6) to 14.6 mmHg (± 4.3). There was a substantial reduction of pulmonary regurgitation from before (none/trivial n = 0, mild n = 2, mode rate n = 9, severe n = 11) to after PPVI (none/trivial n = 20, mild n = 1). During the short-term follow-up of 5.7 months there was no change in the immediate results. CONCLUSION: PPVI using the Edwards SAPIEN(™) pulmonic valve can be performed safely in a wide range of patients with various diagnoses and underlying pathology of the RVOT and enables the restoration of an adult-size RVOT diameter. Although the immediate and short-term results seem promising, the long-term effects and safety have to be assessed in further clinical follow-up studies.

Embolization of immediate release transcatheter patch for atrial septal defect occlusion: the causes and the pitfalls to avoid.

We report embolization of a bioabsorbable immediate-release patch for closure of atrial septal defect into left atrium with forward movement into and out of the mitral valve during each cardiac cycle. The dislodged device was ultimately explanted by a surgical operation and the defect was closed. We discuss causes and recommend tips to prevent such a serious potential complication. We also suggest some modifications to the device for making it more safe and effective.