Background : Carotid artery (CA) access allows a more straight route for many left heart lesions. This has previously been achieved via a surgical cut-down approach in certain pediatric cardiac interventions. However, there are little data considering CA access in pediatric cases, percutaneously. Aim : We hypothesized that there would be notable improvements in efficiency as well as overall success when using the CA for access in selected cases. Methods : Between November 2016 and January 2019, records of patients undergoing attempted percutaneous CA access under ultrasound guidance for cardiac catheterization were reviewed. Results : Thirty patients underwent 36 catheterizations; median age 17 days (range, 6 days–9 months) and median weight 3.2 kg (1.2–7.8). Procedures performed were stenting or stent redilatation of the patent ductus arteriosus in 23, stenting or angioplasty of modified Blalock–Taussig shunts in four, aortic valvuloplasty in three, angioplasty for coarctation of the aorta in four, renal angioplasty in one, and diagnostic catheterization in one case. The intended intervention was unsuccessful in two patients despite successful CA access. Follow-up imaging showed a normal carotid in 28 of 30 (94%), with mild luminal narrowing with normal Doppler velocities in two instances. No patient had clinically apparent neurological sequelae attributable to CA access. Conclusions : Our data indicate that CA access should be employed when dealing with a select group of infants requiring vertical approach for left-sided cardiac lesions. Percutaneous CA access, even in very small preterm infants, is safe and feasible with negligible vascular injury and no neurological adverse events.

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Balloon angioplasty may be performed as the first treatment of aortic coarctation to stabilize newborns too sick for immediate surgery. The issue of vascular access is the key to the successful treatment of critical newborns. In our study, we argue that the lesser-known axillary access route is the safest and most effective route of vascular access for balloon angioplasty in infants with aortic coarctation. To support this argument, we present the case of eight unstable newborns with complex heart diseases, who were successfully treated with percutaneous intervention through the axillary artery. This case series is followed by an analysis of the greater efficacy of this technique compared to the more conventional femoral and carotid routes. We conclude by acknowledging the substantial advantages of this lesser-known vascular access and advocate its more widespread clinical implementation in the treatment of critical newborns.

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Objectives : The main objective of the study is to characterize the effects of genetic abnormalities/syndromes (GA/S) on perioperative outcomes of cardiac surgeries involving repair of conotruncal heart defects (CTHD). Design : The study involves a single-center retrospective analysis of patients who underwent complete repair of CTHDs (tetralogy of Fallot [TOF], truncus arteriosus, interrupted aortic arch, and ventricular septal defect with coarctation) between January 2000 and December 2015. The primary outcome was the post operative length of stay (PLOS). The secondary outcomes were mortality, cardiac complications, hematologic complications, infections, and number of medications-at-discharge. Setting : Cardiac intensive care unit in a tertiary pediatric hospital in South Florida that performs around 300 open-heart surgeries a year. Subjects : A total of 177 patients with CTHDs who underwent cardiac surgeries in the stated time period were included in the final study cohort. Measurements and Main Results :Majority of patients had TOF (72.5%) and 46 (26%) had GA/S. The most common GA/S was 22q11 deletion (37%). PLOS was significantly increased in patients with GA/S (P < 0.05). Patients with GA/S were 4.5 times more likely to have a postoperative cardiac complication, 4.2 times more likely to have a postoperative infection, and received 1.6 times more medications at discharge than those without GA/S. However, GA/S was not associated with increased perioperative mortality. Black patients were three times more likely to have a longer PLOS than White patients. Conclusions : Perioperative outcomes in patients with GA/S suggested an increased residual cardiovascular disease and increased resource usage. Notably, this is the first study demonstrating the effect of race and ethnicity on PLOS in CTHD patients.

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Background: Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was once the procedure of choice for critical PS; however, balloon pulmonary valvuloplasty (BPV) has now become the standard treatment. Although the procedure is usually simple, crossing the pulmonary valve from the femoral vein can be difficult, especially when severe tricuspid regurgitation and right atrium dilatation are present. In such patients, the maneuver can be simplified by using the right internal jugular vein approach. However, many operators are reluctant to use this approach because of unfamiliarity with the technique, potential complications, and paucity of reports. Until now, there is no literature describing BPV using the transjugular approach in neonates, also none directly comparing the transfemoral and transjugular approaches. Objective: We compared transjugular with the transfemoral approach in terms of procedure time and complications. Materials and Methods: This was a retrospective cohort study. Participants were neonates with critical PS undergoing BPV in the National Cardiovascular Center Harapan Kita from 2013 to 2018. Results: Of 15 neonates undergoing BPV, eight were done using the transjugular approach and seven using the femoral approach. Mean age and weight in both groups was similar. In all eight patients using transjugular approach, crossing the pulmonary valve was consistently quick and easy. The total procedural time, pulmonary crossing time, and fluoro time was significantly shorter using the transjugular approach (65 ± 8 vs. 108 ± 17.8 min, P < 0.05; 22 ± 3.1 vs. 45 ± 14.8 min, P < 0.01; 29 ± 13 vs. 67 ± 35 min, P < 0.05). There were no complications relating to vascular access. Moreover, the BPV procedure itself demonstrated comparable results in both groups. Conclusion: BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.

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Objective: The purpose of this review is to present the available literature on the use of nonopioid analgesics such as nonsteroidal anti-inflammatory drugs in postcardiac surgery pediatric patients, mainly to focus on patients <1 year of age, and to provide the foundation for future research. Materials and Methods: Published studies that address the use on nonopioid medications for postoperative sedation and analgesia in infants and children undergoing cardiac surgery were identified from online sources. Studies were reviewed by two authors independently to assess the quality of the data as well as the evidence. Due to limited availability of such studies, the review was then expanded to include use in noncardiac procedures as well as to expanded age groups. All studies that met the primary objective were included. Results/Data Synthesis: Majority of the studies in the population of interest were related to use of ketorolac. Five studies specifically addressed ketorolac use in cardiac patients. In addition, studies were reviewed for nonopioid analgesia in noncardiac patients and included as a part of the available evidence as in the case of acetaminophen use. Newer agents as well as agents with very limited information were also acknowledged. Conclusion: Nonopioid medications appear to show promise for analgesia in infants undergoing cardiac surgery, with ketorolac being the most potent agent as a potential substitute for opioids. These agents demonstrate a reasonable safety profile even in the very young. There continue to be significant gaps in knowledge before their adoption becomes routine. However, gives the awareness regarding short-term and long-term impact of opioid use in this vulnerable population, and studies of such agents are an urgent need.

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This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure. The historical aspects, technical considerations, and results are briefly elucidated.

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Total Anomalous Pulmonary Venous Connection (TAPVC) is frequently classified based on the system proposed by Craig, Darling and Rothney. Occasionally variants are reported which do not fit into these classic forms. One such variant is the double drainage of TAPVC where the confluence has connections at two different levels. We report two infants with a double drainage pattern of TAPVC

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Complete sternal cleft is a very rare congenital midline defect of the sternum. It is not uncommonly associated with intracardiac defects. We report a case of a 2-year-old child with complete sternal cleft and tetralogy of Fallot who presented with cyanotic spells. The child underwent total correction, followed by chest wall reconstruction on the next day.

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Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of the mitral valvar orifice has previously been reported in the setting of mirror-imaged atrial arrangement. We report a neonate with unguarding of the mitral valvar orifice in the setting of usual atrial arrangement, but with discordant atrioventricular connections and pulmonary atresia.

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Background and Objectives: Device closure of secundum atrial septal defect is shown to be feasible and effective in children weighing ≤10 kg. Issues such as how large is too large, how to choose device size, does the length of the interatrial septum (IAS) matter, and need for technical modifications for successful device delivery have not been systematically addressed. Materials and Methods: This is a retrospective study, comprising 45 patients weighing ≤10 kg, who were chosen for device closure between January 2010 and June 2018. Patient selection was done on basis of transthoracic echocardiography. Device closure was done using Amplatzer septal occluder. The device size was selected primarily based on transesophageal echocardiography (TEE)-measured defect diameter. Although IAS length was taken into consideration, adequate rim size was the key factor in deciding device closure of the defect. Results: Forty-three out of 45 patients had successful device closure. The mean age and weight were 25.71 ± 8.62 months and 8.99 ± 1.24 kg, respectively. The defect measuring as large as 27 mm (14.89 ± 3.89) on TEE was closed and device as big as 28 mm was successfully deployed (16.7 ± 4.31). Regular technique of device deployment was successful in only 15 cases. In the remaining 28, one of the modified techniques was used. There was no mortality, failure of the procedure, device embolization, thromboembolism, or pericardial effusion. One patient developed moderate mitral regurgitation and two patients had transient atrioventricular block. At follow-up, all patients showed significant improvement in symptoms and growth without any complications. Conclusions: Defect size as large as three times the weight in kg can be closed in small children. Devices as large as 28 mm can be deployed in these hearts provided the surrounding rims are adequate. In majority of cases, one of the modified techniques is essential for successful deployment. IAS length is not a limiting factor for deciding the size of the device used.

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Objective :The objective of this study is to establish normal reference ranges for the left ventricular mass (LVM) and LVM index (LVMI) in preterm infants according to the body surface area (BSA) and assess their correlation with body weight and gestational age. Subjects and Methods :In a prospective study, 268 preterm babies who fulfilled the criteria for inclusion were examined. Echocardiograms were performed to measure the LVM and LVMI on 0–6 day (s) of life and at weekly intervals until the babies reached 36 weeks. The preterm infants were divided into six groups according to their BSA: 0.07–0.08 m2, 0.09–0.10 m2, 0.11–0.12 m2, 0.13–0.14 m2, 0.15–0.16 m2, and 0.17–0.19 m2. Results : The mean gestational age was 29.8 (±2.38 standard deviation [SD]) weeks, ranging from 24 to 35 weeks. The mean body weight was 1479 (±413 SD) g, ranging from 588 to 3380 g, and the mean BSA was 0.13 m2, ranging from 0.07 to 0.19 m2. The LVM correlated well with the gestational age, body weight, and BSA. The LVMI correlated well with body weight and BSA. Reference ranges with the mean ± SD, range, and interquartile range were calculated for the LVM and LVMI according to the BSA. A significant gradual increase was observed in a LVM with increasing BSA. Overall, a progressive and significant increase in the LVM was observed during the first 9 weeks of life. Conclusion :The LVM and LVMI exhibited a significant correlation with the BSA and body weight. This study provides reference data that can be used as a normal reference tool for the LVM and LVMI for preterm infants based on the BSA.

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Background: The past two decades have seen rapid development of new surgical techniques for repair as well as palliation of complex congenital heart diseases. For a better patient outcome, minimal postoperative ventilation remains one of the most important endpoints of an effectual perioperative management. Aims and Objectives: The aim of this randomized open-label trial was to compare postoperative extubation time and intensive care unit (ICU) stay when two different anesthetic regimens, comprising of induction with ketamine and low-dose fentanyl versus high-dose fentanyl, are used, in pediatric patients undergoing corrective/palliative surgery. Materials and Methods: Patients with congenital cardiac defects, under 14 years of age undergoing cardiac surgery under cardiopulmonary bypass (CPB) and epidural analgesia, were enrolled into two groups – Group K (ketamine with low-dose fentanyl) and Group F (high-dose fentanyl) – over a period of 10 months, starting from January 2018. The effect of both these drugs on postoperative extubation time and ICU stay was compared using Mann–Whitney U-test. Results: A total of 70 patients were assessed with equal distribution in both the groups. In Group K, 32 of 35 patients were extubated in the operation room, whereas extubation time in Group F was18.1 ± 11 h. Total ICU stay in Group K and Group F was 45.2 ± 30.1 and 60.1 ± 24.5 h, respectively (P = 0.02). Systolic blood pressure was significantly higher in Group K. Conclusion: Ketamine along with low-dose fentanyl, when used for anesthetic induction, in comparison to high-dose fentanyl, reduces postoperative extubation time and ICU stay, in pediatric patients undergoing corrective/palliative surgery under CPB and epidural analgesia for congenital cardiac defects.

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The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.

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A 21-day-old baby with transposition of the great arteries with intact ventricular septum, infradiaphragmatic totally anomalous pulmonary venous connection, and atrial septum defect underwent combined arterial switch operation, totally anomalous venous connection repair, and atrial septum defect closure, using a right-sided approach and temporary pulmonary veins occlusion, with no postoperative and 6-months follow-up complications. Complete anatomical correction is the most conceivable treatment for this unusual pathology; right-sided approach instead lifting the heart toward the right pleural cavity to perform left atrium-to-pulmonary veins anastomosis limits heart displacement and avoids nonphysiological three-dimensional alterations; moreover, ligation and division of vertical vein allow to obtain more tissue for anastomosis; temporary occlusion of pulmonary veins while performing anastomosis is a simple procedure that allows to avoid deep hypothermic circulatory arrest or low flow systemic perfusion. Combination of these details facilitates intra- and postoperative management, especially in combined demanding cases.

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Superior vena cava (SVC) and inferior vena cava obstructions were once well-documented complications from the Mustard repair for D-transposition of the great arteries (TGA), occurring in 10%–40% patients; however, they are rarely documented with the current, more common arterial switch operation (ASO). Similarly, SVC thrombosis is an uncommon, severe complication following neonatal cardiac surgery. We report a case of persistent SVC thrombosis, SVC syndrome, and chylothorax arising after ASO, refractory to thrombolysis and stent placement. A 6-day-old neonate with prenatally known TGA underwent an arterial switch procedure. Despite an initially unremarkable postoperative course, he developed respiratory difficulty after starting enteral feeding. Soft-tissue swelling was noted in the neck, chest, and upper face. An SVC thrombus was identified with cardiac catheterization. Multiple thrombolytic modalities were attempted. His postoperative course was further complicated by recurrent chylothoraces, respiratory failure, sepsis, anasarca, and renal failure. He was eventually transferred to a larger center for a special lymphatics evaluation, where two lymphovenous anastomoses were unsuccessful. He was sent to his home hospital, where he died from extended-spectrum beta-lactamase Klebsiella sepsis. Early diagnosis of SVC syndrome and prompt thrombolysis may prevent the complications encountered in this patient. More research is needed in neonatal thrombolysis and anticoagulation.

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Arterial dissections are uncommon in neonates. Complications include thrombosis, bleeding, dissection, aneurysm and pseudoaneurysm. We report an unusual case of multisite pathology (dissection and pseudoaneurysm) following common vascular interventions. A term neonate with antenatal diagnosis of congenital heart block secondary to maternal lupus deteriorated clinically at 5 days of life. He was found to have an abdominal aortic thrombus secondary to abdominal aortic dissection, following umbilical arterial catheter placement. Attempted percutaneous treatment was complicated by dissection of the left common carotid artery and formation of a large pseudoaneurysm. Neonatal lupus is associated with weakened vessel wall which may be vulnerable to injury from line placement and endovascular interventions. Various options are available to manage arterial dissection, thrombus, and pseudoaneurysm, but consequences of these options need to be carefully weighed to minimize further complications.

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The persistence of the fifth aortic arch (PFAA) in postnatal life is an extremely rare and controversial cardiovascular malformation. PFAA is defined as an extra-pericardial vessel arising from the ascending aorta proximal to the origin of the brachiocephalic arteries, terminating either in the dorsal aorta or in the pulmonary arteries through the persistently patent arterial duct. An isolated PFAA with systemic-to-pulmonary connection best fits this definition, while the vast majority of cases reported as PFAA may have alternative embryological explanations. We present a unique case of a 5-week-old patient with an isolated PFAA with systemic-to-pulmonary connection, who presented with congestive heart failure. A first differential diagnosis was made with distal aortopulmonary window and an atypical patent arterial duct. A careful analysis of the case and a systematic review of the literature made us conclude for an isolated PFAA, which is one of the only five cases ever reported.

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Congenital mitral stenosis (MS) is a spectrum of anomalies that result in functional and anatomic obstruction of inflow into the left ventricle. Mitral arcade is one of the varieties of congenital MS where there is an abnormal development of chordae tendineae, resulting in stenosis, regurgitation, or both. Here, we describe the case of a mitral arcade in a child, which was diagnosed on echocardiography and confirmed with other imaging modalities.

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A circumflex retroesophageal left aortic arch with a right-sided ductus is an extremely rare cause of a complete vascular ring, which may result in severe tracheobronchial compression, leading to respiratory compromise, especially in children. We present a case of a 6-month-old female child with stridor and feeding difficulties since birth with interspersed self-resolving episodes of cyanosis and apnea, secondary to the presence of the above-mentioned vascular ring.

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