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Turner syndrome (TS) is a genetic disorder presenting in phenotypic females with total or partial monosomy of the X chromosome. Cardiovascular abnormalities are common, including congenital heart defects (CHD) and aortic dilation. Although mosaic TS is suspected to have less severe phenotype as compared to non-mosaic TS, differences in cardiovascular manifestations between karyotypes are not well studied. This is a single-center retrospective cohort study including patients with TS seen from 2000 to 2022. Demographic data, chromosomal analysis, and imaging were reviewed. Karyotypes were categorized as monosomy X (45X), 45X mosaicism, isochromosome Xq, partial X deletions, ring X (r(X)), TS with Y material, and others. Prevalence of CHD and aortic dilation were compared between monosomy X and other subtypes using Pearson's chi-square test and Welch two-sample t-test. We included 182 TS patients with median age 18 (range 4–33) years. CHD was more common in monosomy X as compared with others (61.4% vs. 26.8%, p < 0.001), including bicuspid aortic valve (44.3% vs. 16.1%, p < 0.001), partial anomalous pulmonary venous return (12.9% vs. 2.7%, p = 0.023), persistent left superior vena cava (12.9% vs. 1.8%, p = 0.008), and coarctation of the aorta (20.0% vs. 4.5%, p = 0.003). Cardiac surgery (24.3% vs. 8.9%, p = 0.017) was more prevalent in the monosomy X group. There was no statistically significant difference for presence of aortic dilation (7.1% vs 1.8%, p = 0.187). Although CHD and need for cardiac surgery are more common in TS with monosomy X as compared to others, all TS subtypes may have similar risk of developing aortic dilation. All TS patients should have similar cardiovascular surveillance testing to monitor for aortic dilation. [ABSTRACT FROM AUTHOR]

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m2, p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO2/kg/min: R: − 0.45, p = 0.01 peak, minute ventilation (VE) R: − 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population. [ABSTRACT FROM AUTHOR]

As a strategy for the primary Norwood operation, the right ventricular–pulmonary artery shunt is associated with satisfactory early outcome. However, use of this shunt after bilateral pulmonary artery banding remains controversial. This study compared the operative outcomes and late hemodynamics in patients who underwent the Norwood operation, preceded by bilateral pulmonary artery banding, with a right ventricular–pulmonary artery shunt or with bidirectional Glenn anastomosis (comprehensive stage II strategy). We retrospectively reviewed 38 patients who underwent the Norwood operation preceded by bilateral pulmonary artery banding between 2004 and 2017. Of these, 17 underwent the Norwood operation with a right ventricular–pulmonary artery shunt (Group S), whereas 21 underwent the comprehensive stage II strategy (Group G). 5 years after the Norwood operation, 10 (60%) and 17 (81%) patients in Group S and Group G, respectively, underwent the Fontan procedure. Group S showed significantly lower pressure in the superior vena cava after bidirectional Glenn anastomosis than Group G (13 ± 2 mmHg vs. 18 ± 3 mmHg; p < 0.01), but pressures were similar after the Fontan procedure. The right ventricular end-diastolic volume at 1 year post-Fontan procedure was significantly higher in Group S than in Group G (142 ± 41% vs. 91 ± 28%; p < 0.01). In terms of early outcomes, the Norwood operation with a right ventricular–pulmonary artery shunt enabled low pressure in the superior vena cava, but in the long term, this shunt adversely influenced the right ventricular volume. [ABSTRACT FROM AUTHOR]

To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation. [ABSTRACT FROM AUTHOR]

Persistent left superior vena cava (PLSVC) draining to the left atrium (LA) is a rare congenital abnormality that is often asymptomatic and found incidentally on imaging. PLSVC is usually described alongside other congenital defects, such as septal defects, tetralogy of fallot, and aortic coarctation. PLSVC to LA with an atrial septal defect is known as Raghib syndrome, but to our knowledge PLSVC to LA without an atrial septal defect or right superior vena cava has not been described in the literature. Here, we report the presentation of a patient with PLSVC-LA without ASD and propose a classification system for this subset of congenital heart defects to help guide clinical and surgical management of these patients. [ABSTRACT FROM AUTHOR]

This study aims to define the associated anomalies with PLSVC, and to compare single PLSVC and bilateral superior vena cava in terms of accompanying anomalies and pregnancy outcomes. This was a retrospective study of the fetuses diagnosed with single and/or bilateral SVC at a tertiary fetal medicine center during 8 years. We detected 16 cases of single PLSVC and 84 cases of bilateral SVC. We found an association between the PLSVC and cardiac and extracardiac anomalies. Comparison between single PLSVC and BSVC cases revealed significant differences in the occurrence of heterotaxy and right isomerism. The study highlights the importance of prenatal diagnosis in PLSVC cases. Isolated PLSVC with situs solitus may be considered a benign finding, but larger studies are needed to understand the clinical implications of PLSVC in relation to chromosomal anomalies. Routine screening protocols should include three-vessel and trachea views to detect PLSVC. [ABSTRACT FROM AUTHOR]

This article discusses the use of virtual reality (VR) software in aiding the precise closure of sinus venosus defects using covered stents without the need for 3D printing. The article explains that complex stenting procedures require precise positioning and expansion of stents, which can be achieved through VR simulations. The case report presented in the article describes the successful closure of a sinus venosus defect using a covered stent, with guidance from computed tomographic images and the VMersive VR platform. The article concludes by acknowledging the introduction of the VMersive platform to the department and stating that there are no competing interests or funding involved. [Extracted from the article]

Device closure has become the preferred procedure for treating oval fossa defects in the last two decades. More recently, transcatheter sinus venosus defect (SVD) closure has emerged as an alternative to surgery. Transcatheter stenting aims to overcome potential late surgical complications such as stenosis of the superior vena cava (SVC) and right upper pulmonary vein (RUPV), as well as sinus node dysfunction. Balloon interrogation of the cavoatrial junction is able to identify patients who are suitable candidates for nonsurgical closure. Successful closure is possible when the balloon seals the SVD and redirects the RUPV towards the left atrium. Oval fossa (secundum) defects can coexist in approximately 9–16% of patients with SVD. Among a group of 80 patients who underwent transcatheter closure of SVD, five adult patients aged between 22 and 52 years also required device closure of an associated oval fossa defect. The procedure involved simultaneous balloon interrogation of both the SVD and oval fossa defect, with continuous monitoring of the RUPV using bilateral femoral venous sheaths. Covered stent exclusion of the SVD was performed with concurrent device closure of the oval fossa defect using 12–36 mm atrial septal occluders. During the procedure, two patients required protective balloon inflation in the RUPV while expanding the covered stent. In one patient, a higher small accessory RUPV was intentionally left to drain into the SVC through the struts of a bare stent anchoring the covered stent in the upper SVC. In another patient, a second overlapping covered stent was used to address residual flows from a fabric tear that became apparent after balloon deflation. There were no vascular complications and only one patient exhibited an insignificant 6 mm residual flow from the caudal edge of the SVD during a follow-up of 5 to 72 months. In conclusion, the closure of both SVD and associated oval fossa defects can be successfully performed in a single procedure, with comparable procedural times and favourable mid-term outcomes. [ABSTRACT FROM AUTHOR]

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM. [ABSTRACT FROM AUTHOR]

A patient was delivered at 26 weeks (about 6 months) gestation via an emergency caesarian section. A patent ductus arteriosus (PDA) and atrial septal defect (ASD) were discovered during an echocardiogram 13 days after birth. The patient had catheter-based closure of the PDA and ASD. During a routine echocardiogram to check device placements, it was discovered that there was dilation of the superior vena cava (SVC), and it was suspected that a thrombus was present. Computed tomography angiography (CTA) was completed to better define SVC anatomy and flow acceleration. The CTA demonstrated that there was a double innominate vein. [ABSTRACT FROM AUTHOR]

Invasive and non-invasive monitoring allow for early detection of hemodynamic compromise, facilitating timely intervention and avoidance of further decline. While venous oximetry is useful for assessing the adequacy of systemic oxygen delivery (DO2), it is most often intermittent, invasive, and costly. Near-infrared spectroscopy (NIRS) oximetry allows for the non-invasive estimation of the adequacy of DO2. We assessed the correlation between cerebral NIRS oximetry and superior vena cava (SVC) and jugular venous (JV) oxygen saturations and between renal NIRS oximetry and inferior vena cava (IVC) oxygen saturations. Systematic review of the literature was conducted to identify studies with data regarding near-infrared spectroscopy and venous saturation. The PubMed, EMBASE, Medline, and Cochrane databases were queried using the following terms in isolation and various combinations: "congenital heart disease," "near infrared spectroscopy," "venous saturation," and "pediatric." Pediatric studies in which simultaneous NIRS oximetry and corresponding venous oxygen saturations were simultaneously collected after cardiac surgery or catheterization were identified. Data were pooled from these studies to analyze the correlation between NIRS oximetry and the corresponding venous oxygen saturations. A total of 16 studies with 613 patients were included in the final analyses. Data were present to compare cerebral and renal NIRS oximetry with corresponding venous oxygen saturation. Cerebral NIRS and SVC and JV oxygen saturations and renal NIRS and IVC oxygen saturations demonstrated strong degrees of correlation (r-value 0.70 for each). However, cerebral NIRS and IVC oxygen saturation had a week degree of correlation (r-value of 0.38). Pooled analyses demonstrate that cerebral NIRS oximetry correlates strongly with SVC or JV oxygen saturation while renal NIRS oximetry correlates strongly with IVC oxygen saturations. A weak correlation was noted between cerebral NIRS oximetry and IVC oxygen saturations. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population. [ABSTRACT FROM AUTHOR]

Arrhythmia is related to heart rate variability (HRV), which reflects the autonomic nervous regulation of the heart. We hypothesized that autonomic nervous ganglia, located at the junction of the superior vena cava's entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV. We aimed to investigate changes in heart rate and HRV in a cohort of children with univentricular heart defects, undergoing stepwise surgery towards total cavopulmonary connection (TCPC), and compare these results with healthy controls. Twenty four hours Holter-ECG recordings were obtained before BDG (n = 47), after BDG (n = 47), and after total cavopulmonary connection (TCPC) (n = 45) in patients and in 38 healthy controls. HRV was analyzed by spectral and Poincaré methods. Age-related z scores were calculated and compared using linear mixed effects modeling. Total HRV was significantly lower in patients before BDG when compared to healthy controls. The mean heart rate was significantly reduced in patients after BDG compared to before BDG. Compared to healthy controls, patients operated with BDG had significantly reduced heart rate and reduced total HRV. Patients with TCPC showed reduced heart rate and HRV compared with healthy controls. In patients after TCPC, total HRV was decreased compared to before TCPC. Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events. [ABSTRACT FROM AUTHOR]

Bilateral superior vena cava (SVC), which occurs following bilateral bidirectional cavopulmonary shunt (BCPS), is an anomaly marked by unique hemodynamics. This study aimed to determine its effects on outcomes after Fontan completion. Among 405 patients who underwent BCPS and total cavopulmonary connection (TCPC) between 1997 and 2017, 40 required a bilateral-BCPS. The dominant SVC prior to TCPC was identified according to the direction of blood flow in the central pulmonary artery, and its relationship to the inferior vena cava (IVC) was classified as a concordant or discordant relationship. Preoperative factors were analyzed to identify the risk factors for specific adverse outcomes. The length of intensive care unit (ICU) stay after TCPC was longer in the 40 patients who underwent bilateral-BCPS than in those who underwent unilateral BCPS (p = 0.024), and the survival rate was lower in the former group than in the latter group (p = 0.004). In the patients who underwent bilateral-BCPS, the dominant SVC was concordant with the IVC in 30 patients and discordant in 10 patients. With regard to whether certain morphological, hemodynamic, and flow dynamics-related variables were risk factors for adverse outcomes following TCPC, a discordant relationship between dominant SVC and IVC was identified as an independent risk factor for both a longer ICU stay (p = 0.037, HR 2.370) and worse survival (p = 0.019, HR 13.880). Therefore, in patients with a bilateral SVC who have previously undergone bilateral-BCPS, a discordant relationship between dominant SVC and IVC might contribute to worse outcomes following TCPC. [ABSTRACT FROM AUTHOR]

To analyze the prevalence, types, and risk factors for cardiac arrhythmias associated with peripherally inserted central catheters (PICCs) in children. This is a case–control single center retrospective study. From 3180 PICCs inserted in children (< 18 years old) between 2009 and 2013, cases with new onset arrhythmias were identified. Demographics, type and timing of arrhythmias, and possible risk factors were analyzed. ECGs, rhythm strips, physicians' records, and anti-arrhythmic management were confirmatory. The level of the PICC's tip in the superior vena cava (SVC) or right atrium (RA) was ascertained from chest X-rays and counted in rib units (RU) and vertebra units (VU). Cases were matched (1:1 ratio) to controls by weight and date of insertion. Descriptive statistics were performed. A two-sided p value < 0.05 was considered significant. Thirty-one children (1%) developed arrhythmias, 16/31 (56%) were males, and 24/31 (77%) were < 1-year age. Arrhythmias were atrial 22 (71%), ventricular 4 (13%), and undetermined 5 (16%). Median PICC dwell time was 16 days. 14/31 (45%) cases and 9/31 (29%) controls had underlying structural heart disease. PICCs central tip position was lower among cases than controls (RU 6 vs 5). Odds Ratio for developing arrhythmia was 4.5 (95% CI 0.98–20.83) if the tip lays below 6.25RU. Arrhythmias were resolved with anti-arrhythmic agents in 52% (16/31) and with PICC exchange/manipulation in 32% (10/31) cases. Two children died unrelated to arrhythmia. Prevalence of arrhythmias associated with PICCs in children is low (1%). Arrhythmias are 4.5 times more likely when PICC's central tip position is deeper than 6.25RU. [ABSTRACT FROM AUTHOR]

The frequency of chromosomal anomalies among fetuses with isolated persistent left superior vena cava (PLSVC) is still debated. The objective of the present study was to assess the prevalence of genetic and morphological anomalies identified in fetuses with PLSVC. We conducted a single-center retrospective study including all fetuses diagnosed with a PLSVC between 2010 and 2017. PLSVC was categorized as isolated or associated according to antenatal diagnosis of associated congenital heart defects, hypoplastic aortic isthmus, abnormal venous/arterial connections, and extracardiac anomalies. Among 229 fetuses diagnosed with PLSVC, 39 cases (17%) were strictly isolated and no syndromic/genetic anomaly or aortic coarctation was diagnosed. Seventy-two fetuses had a cardiovascular defect with a rate of genetic anomalies of 22%, 29 had an extracardiac malformation, and 89 had both an extracardiac and a cardiovascular defect. Among fetuses with abnormal development of the arterial/venous system as the only associated anomaly such as aberrant right subclavian artery or absent ductus venosus, 22% had a genetic anomaly. Overall, sixty-five fetuses or infants had a genetic concern, including 23 aneuploidies, 15 pathogenic micro-deletions/duplications, and 5 variants of unknown significance; 12 patients had VACTERL association, and 12 heterotaxy syndrome. Seven infants had an aortic coarctation diagnosed at birth. In conclusion, a thorough prenatal ultrasound examination is paramount, and the identification of variants of the venous/arterial system in addition to PLSVC should raise suspicion for genetic or morphologic abnormalities. Invasive prenatal diagnosis with array-CGH should be offered when PLSVC is non-isolated, after a detailed ultrasound evaluation in a tertiary center. [ABSTRACT FROM AUTHOR]

Partial anomalous pulmonary venous drainage (PAPVD) is a common congenital heart disease. If the insertion of an anomalous pulmonary vein (PV) is high into the superior vena cava (SVC), the traditional 1-patch or 2-patch surgical repair might be challenging. Unlike patch procedures, the cavoatrial anastomosis technique (Warden procedure) theoretically reduces complications such as sinus node dysfunction and venous obstruction. We investigate outcomes of the Warden procedure (WP) at a single institution. A total of 67 patients (42 males and 25 females) with PAPVD who underwent the WP between January 2011 and December 2018 were consecutively enrolled. The median age was 2.8 years (3–61 years old). 52 cases were younger than 18 years old. perioperative and follow-up clinical data were collected. In addition, we selected possible risk factors (a total of 18 risk factors) of SVC stenosis or obstruction. Univariate and multivariate analyses were conducted to confirm the independent risk factors. The average Cardiopulmonary bypass (CPB) time was 132.3 ± 46.3 min, and the aortic cross-clamp time was 85.2 ± 35.7 min. One patient died postoperatively in hospital. The mean follow-up time of the remaining patients (n = 66) was 15.8 ± 14.5 months (3–64 months). No abnormal cardiac function or sinus node dysfunction cases were identified during the follow-up. Of the survival patients, no venous obstruction was presented before the discharge. Numbers of patients identified with mild PV and SVC stenosis were 1 (1.5%) and 7 (10.6%, 4 of them turned negative eventually) at discharge. At the last follow-up, no PV obstruction was identified, whereas, 4 cases (6%) had SVC obstruction. Numbers of PV and SVC stenosis cases were 3 (4.5%) and 8 (12%). Univariate and multivariate analyses showed that only the diameter of SVC less than 10 mm was an independent risk factor for SVC stenosis or obstruction at the last follow-up. Warden procedure can effectively treat PAPVD (anomalous drainages into SVC), with satisfactory early and mid-term postoperative results. Different modifications of the Warden procedure may be selected according to the anatomic characteristics. The diameter of SVC less than 10 mm predicts SVC stenosis or obstruction after Warden procedure. [ABSTRACT FROM AUTHOR]

Transfemoral and right transjugular approach has been described for transcatheter pulmonary valve implantation (TPVI). However, the use of left transjugular approach through the left superior vena cava to coronary sinus has not been well described. We present a 22-year-old male with Tetralogy of Fallot status post transannular patch, who underwent successful TPVI using the Sapien valve, preceded by pre-stenting of native right ventricular outflow tract. All the transcatheter intervention was performed through the left superior vena cava to coronary sinus. [ABSTRACT FROM AUTHOR]

Furthermore, Fick's equation to calculate cardiac output equates cardiac output to the quotient of oxygen consumption and the arteriovenous oxygen content difference. Often, critics of near infrared spectroscopy will cite large limits of agreement between near infrared spectroscopy and corresponding regional venous saturations as a limitation in the utility of near infrared spectroscopy [[2]]. We applaud the recent publication by Spaeder and colleagues in which they describe their implementation of a thought model pertaining to the use of near infrared spectroscopy. [Extracted from the article]

Risk of superior vena cava (SVC) obstruction following repair of anomalous right upper pulmonary veins in children is unclear. The incidence and outcome of subclinical obstruction remained unknown. Retrospective single institutional study (07/1993–02/2017) in a pediatric population (N = 42, median age 3.9-year, range 0.1–15.3 years). 33 (79%) children had repair without SVC translocation ("non-Warden") and 9 (21%) had Warden-type surgery. Echocardiographic SVC obstruction was defined as (I) turbulent flow across SVC and (II) continuous flow pattern without return to baseline velocity (0 m/s); severe obstruction was defined as loss of distinct biphasic profile ± mean gradient ≥ 5 mmHg. 3 (7%) patients required intra-operative revision due to obstruction (non-Warden: 1, Warden: 2). After discharge, 2 (5%) patients required reintervention (3 and 6-month post-op) for severe symptomatic obstruction (non-Warden: 1, Warden-type: 1). Both patients responded to balloon angioplasty with symptomatic resolution (one required repeat catheter reintervention). 10 (24%) patients had subclinical echocardiographic obstruction (2, 22% Warden vs. 8, 24% non-Warden; p = 1.0; 8 of 10 patients had mild gradient), which resolved and remained well without reintervention. At follow-up (mean 7.2-year, range 0–23 years), all patients were alive. Freedom from SVC reintervention at 10 and 20-year is 95% (97% at 10, 20-year in non-Warden and 89% at 5, 8-year in Warden-type group; log-rank p = 0.34). Surgical repair for anomalous right upper pulmonary veins is associated with risk of SVC obstruction in children. The need for reintervention for severe obstruction is rare at late follow-up. Patients with subclinical obstruction remain asymptomatic and demonstrate echocardiographic improvement. [ABSTRACT FROM AUTHOR]

To assess the outcomes of neonates prenatally diagnosed with ventricular asymmetry and not operated on within the neonatal period and to determine the risk factors for left heart obstruction occurrence at follow-up. All neonates with prenatal asymmetry of the ventricles, diagnosed from August 1993 to July 2015, not operated on within the neonatal period, were retrospectively included in the study. Left heart echocardiographic measurements at birth and at last follow-up were collected and compared. Left heart anomaly included isthmus and/or aortic valve and/or mitral valve obstruction. There were a total of 34 newborns included in the study. The median follow-up was 2 years. There was no death. Eleven patients were operated on at a median age of three months; seven of them had an obstruction of the left heart (five coarctations of the aorta, one sub-aortic and aortic valve stenosis, and one mitral stenosis). Estimated freedom of left heart surgery was 80% at 6 months and 75% at 10 years. The main risk factor for progression to a left heart anomaly was a hypoplasia of the aortic isthmus (p = 0.0003), while the presence of a left superior vena cava was more frequent in these patients although the difference was not significant. Patients with an aortic isthmus z-score below − 2 at the closure of arterial duct are at risk of later coarctation and therefore follow-up should be extended to at least 3 months. Furthermore, the prenatal ventricular asymmetry does not only identify patients at risk of coarctation but also of other left heart anomalies. This last point should be a better approach with future parents to improve prenatal counseling on a more complex postnatal diagnostic than a simple isolated coarctation. [ABSTRACT FROM AUTHOR]

Anomalous drainage of the right superior vena cava into the left atrium (LA) has been previously reported in the medical literature. We present a unique case of a child who had two superior vena cavae, a left superior vena cava that drained normally through the coronary sinus to the right atrium, and a right superior vena cava that drained anomalously to the LA. [ABSTRACT FROM AUTHOR]

Examples of situs terminology confusion exist in the medical literature, patient medical records, and even collegial discussions. Congenital cardiologists have embraced situs description; nevertheless, except for right isomeric situs, most situses are associated with normal cardiac anatomy B , b including left isomeric situs B , b which often occurs without cardiovascular malformations [[10]]. The simplest method for identifying a patient's situs is by ultrasound of the upper-abdominal inferior vena cava and aorta anatomy and their relationships to each other [[2]]. [Extracted from the article]

To assess a two-phase contrast injection protocol for contrast enhancement during cardiac computed tomography (CT) in children with congenital heart disease. Forty-three children (20 boys, 23 girls) of median age 13 months (range 3 days—8.3 years) and weighing ≤ 20 kg who underwent cardiac CT using a two-phase contrast injection protocol at our institution were retrospectively identified. High-pitch spiral third-generation dual-source cardiac CT (tube voltage 70 kV) was performed with a fixed delay of 60 s after contrast injection in the order of 10 mgI/kg/s (30 s), 15 mgI/kg/s (20 s), and a saline chaser (10 s). Attenuation in the inferior vena cava (IVC), superior vena cava (SVC), right atrium (RA), right ventricle (RV), pulmonary artery (PA), left atrium (LA), left ventricle (LV), and descending aorta (AO) was compared using the Steel-Dwass and Fisher’s exact tests. The median (interquartile range) attenuation in the IVC, SVC, RA, RV, PA, LA, LV, and AO was 285 (264-347) Hounsfield units (HU), 416 (370-445) HU, 368 (320-388) HU, 373 (322-417) HU, 397 (330-432) HU, 425 (373-469) HU, 435 (385-468) HU, and 437 (392-491) HU, respectively (p < 0.05, IVC vs. the other anatomic sites). There was no significant difference in diagnostic success rate for attenuation > 250 HU between the IVC (41 children, 95.3%) and the other sites (43 children, 100%). A two-phase contrast injection protocol is useful for effective contrast enhancement in pediatric cardiac CT. [ABSTRACT FROM AUTHOR]

Evidence suggests an association between left heart obstructive lesions and dilated coronary sinus (DCS), but this has not been studied in fetuses. A retrospective review of fetal echocardiograms (FE) over an 8-year period was conducted, and patients with DCS were identified and confirmed postnatally. There were 5840 FE performed on 4920 women during this period. Of 49 patients with DCS, 22 had normal intracardiac anatomy and 27 patients had congenital heart disease (CHD) yielding an incidence of 4.6 % in the presence of CHD (27/584). Of 27 patients with DCS and CHD, approximately a third had either hypoplastic left ventricles and/or coarctations (10/27, 37 %). The incidence of left heart obstructive lesions was much higher in the presence of a DCS (37 % vs 45/557, 8 %, p < 0.0001). The odds ratio of left heart hypoplasia in fetuses with CHD and a DCS was 6.6 (95 % CI 2.8-15.3). Comparison of patients with postnatally confirmed coarctation with those with normal intracardiac anatomy with DCS, revealed that in the former, the right ventricle ( p = 0.005), pulmonic valve annulus ( p = 0.0001) and the tricuspid inflow were larger ( p = 0.001) compared to corresponding left-sided structures. The size of the DCS was not significantly different between the two groups, but in the former, the DCS was more closely related to the posterior leaflet of the mitral valve and caused a significant diminution of the mitral inflow. Our study suggests a strong association, possibly causal, between left heart obstructive lesions and DCS in utero. [ABSTRACT FROM AUTHOR]

Bone-specific alkaline phosphatase (BALP) is produced by osteoblasts. A recent series noted a positive association between cardiac index (CI) and BALP in patients with Fontan circulation. CI is low at baseline in these patients, and small decreases in CI may result in diverting of blood away from bone. We prospectively enrolled 15 patients (males ≤ 14 yo, females ≤ 12 yo) who had previously undergone Fontan operation and were undergoing cardiac catheterization. Serum BALP was measured at catheterization, and analysis performed to evaluate association between age-/gender-specific BALP z-score and CI as well as other patient variables. The median age at catheterization was 5.6 years (3.1-13.1), and time from Fontan was 1.5 years (0.1-12.1). The median superior vena cava saturation (SVC) was 65 % (52-74), median average between SVC and inferior vena cava (IVC) saturations was 62.5 % (51-70), and median CI was 3.8 L/min/m (2.0-8.4). The median BALP was 65 IU/L and BALP z-score was −2.1 (−3.2 to 0.9). BALP z-score was not associated with CI ( ρ = −0.1, p = 0.7), but a positive correlation was noted with the average of SVC and IVC saturation ( ρ = 0.5, p = 0.052) and with SVC saturation ( ρ = 0.4, p = 0.07), both nearly reaching statistical significance. In our cohort of children with Fontan circulation undergoing catheterization, BALP z-score was not associated with CI, but an association with estimates of mixed venous saturation was noted that nearly reached statistical significance. We hypothesize that BALP is a marker of oxygen delivery in those with Fontan circulation and may represent a valuable biomarker in this population. [ABSTRACT FROM AUTHOR]

Ventricular size discrepancy may be due to a persistent left superior vena cava (PLSVC) in utero. We sought to investigate for differences in cardiac structure measures and hemodynamics between fetuses with isolated PLSVC connected to the coronary sinus (CS) and normal. Fetuses diagnosed with isolated PLSVC in the second and third trimester were enrolled. We defined two groups: group 1, twenty-five fetuses in the second trimester (22-27 W + 6d); group 2, twenty-two fetuses in the third trimester (28-39 W + 6d). Fifty-three fetuses without intra-cardiac or extra-cardiac anomalies and gestation age-matched were divided into normal control groups: group 3, 28 fetuses in the second trimester; group 4, 25 fetuses in the third trimester. Parameters of cardiac structure and hemodynamics were measured, including: left- and right-side heart size, the diameter of foramen ovale, aorta (AO), aortic isthmus and pulmonary artery (PA), and ratios of cardiac structure RV/LV, RA/LA and PA/AO were calculated. Hemodynamic parameters measured included: flow velocity across mitral valve, tricuspid valve and foramen ovale. In the second trimester, the ratio of RV/LV and PA/AO of the PLSVC fetuses was significantly larger than normal, while the AO diameter, aortic isthmus diameter were smaller than normal ( P < 0.05). However, in the third trimester, only the ratio of PA/AO of the PLSVC fetuses was significantly larger, and the aortic isthmus diameter was still smaller than normal ( P < 0.05). Isolated PLSVC connecting to the CS is associated with differences in cardiac structure size from normal. These differences appear to diminish with gestational age. A dilated CS may have an influence on development of fetal left heart structures. [ABSTRACT FROM AUTHOR]

The hemodynamic effects of dexmedetomidine (DEX) on pulmonary artery pressure (PAP) are not fully understood in patients with a single-ventricle physiology. The objective of this retrospective study was to characterize the effect of perioperative DEX infusion on PAP and the transpulmonary pressure gradient after a bidirectional superior cavopulmonary shunt (BCPS) operation. We retrieved physiologic data including the heart rate, incidence of cardiac pacing, systolic and diastolic arterial blood pressure (ABP), and superior vena cava (SVC) and inferior vena cava (IVC) pressures from the medical records of all patients <12 months of age who underwent a BCPS operation. Patients with an additional Norwood or Damus-Kaye-Stansel procedure, those with additional pulmonary blood flow, and those without both a SVC and an IVC catheter were excluded from the present study. Following the BCPS operation, the SVC pressure is equivalent to the PAP. Similarly, the IVC pressure is equivalent to the common atrial pressure (CAP). Accordingly, we can directly assess the transpulmonary pressure gradient, defined as the difference between the PAP and the CAP, using simultaneous SVC and IVC pressure measurements. Twenty-nine patients were included in the present study. We did not find any increase in the PAP, CAP, PAP/systolic ABP ratio, or the transpulmonary pressure gradient as of 6 h after admission to the intensive care unit when the patients were treated with DEX infusion at a median (interquartile ranges) dose of 0.6 mcg/kg/h (0.4, 0.64 mcg/kg/h). We concluded that the administration of DEX to children with a single-ventricle physiology is acceptable. [ABSTRACT FROM AUTHOR]

We present a case of small coronary sinus defect detected after transcatheter device closure of a large secundum atrial septal defect. Although device erosion of the dilated coronary sinus is suspected, the defect in the coronary sinus may have been present prior to ASD device closure. Dilated coronary sinus may be a risk factor when closing a secundum ASD with a device. To the best of our knowledge, coronary sinus erosion by an ASD device has not yet been reported in the medical literature. [ABSTRACT FROM AUTHOR]

This retrospective study aimed to determine the superior vena cava (SVC) and left innominate vein (INV) normative cross-sectional area in children noninvasively using age as a predictor and also to compare the correlation of the area measured with the diameter on multidetector computed tomography (MDCT). Analysis of the SVC-INV cross-sectional area was performed for 73 consecutive patients. The cross-sectional area of the SVC-INV was manually estimated. A regression analysis was performed for the cross-sectional area and age separately, and regression equations were compared. One-way analysis of variance (ANOVA) was performed to evaluate significant differences in the area means according to age groups. Regression analysis showed that age can be a predictor for the area of the SVC (50.6 mm + 1.01 × age), te INV (48.3 mm + 0.93 × age), and the left SVC-INV junction (47.2 mm + 0.92 × age), with respective R values of 93, 88 and 94 %. The comparative evaluation of the cross-sectional area and the diameter measurement of SVC showed that the cross-sectional area was more closely associated with the increasing age of the cohort ( R of 68 vs. 61 %) than the measured diameter. For a cohort of patients without congenital or acquired heart disease, MDCT can be used as a complementary test for a normative cross-sectional normogram area database of SVC-INV using age as a predictor. [ABSTRACT FROM AUTHOR]

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery. [ABSTRACT FROM AUTHOR]

Intraatrial-conduit Fontan is considered a modification of both extracardiac and lateral-tunnel Fontan. In this study, the patient-specific hemodynamic performance of intraatrial-conduit and lateral-tunnel Fontan with fenestration, considered as conversion templates, was investigated based on the authors' patient cohort. Pulsatile computational fluid dynamics simulations were performed using patient-specific models of intraatrial-conduit and lateral-tunnel Fontan patients. Real-time 'simultaneous' inferior and superior vena cava, pulmonary artery, and fenestration flow waveforms were acquired from ultrasound. Multiple hemodynamic performance indices were investigated, with particular focus on evaluation of the pulsatile flow performance. Power loss inside the lateral-tunnel Fontan appeared to be significantly higher than with the intraatrial-conduit Fontan for patient-specific cardiac output and normalized connection size. Inclusion of the 4-mm fenestration at a 0.24 L/min mean flow resulted in a lower cavopulmonary pressure gradient and less time-averaged power loss for both Fontan connections. Flow structures within the intraatrial conduit were notability more uniform than within the lateral tunnel. Hepatic flow majorly favored the left lung in both surgical connections: conversion from lateral-tunnel to intraatrial-conduit Fontan resulted in better hemodynamics with less power loss, a lower pressure gradient, and fewer stagnant flow zones along the conduit. This patient-specific computational case study demonstrated superior hemodynamics of intraatrial-conduit Fontan over those of lateral-tunnel Fontan with or without fenestration and improved performance after conversion of the lateral tunnel to the intraatrial conduit. The geometry-specific effect of the nonuniform hepatic flow distribution may motivate new rationales for the surgical design. [ABSTRACT FROM AUTHOR]

The Amplatzer septal occluder is commonly used to close secundum atrial septal defects (ASDs). Recently, the manufacturer's recommendations have been modified with regard to contraindications for its use. These changes primarily focus on evaluation of the aortic rim (anterio-superior rim) with transesophageal echocardiography (TEE) and intracardiac echocardiography (ICE). In this editorial, we will review systematically all of the sequential views in TEE and ICE that are required for thorough assessment of the aortic rim and provide explanation of what constitutes deficiency of the aortic rim as seen on ICE. [ABSTRACT FROM AUTHOR]

Ellis-van Creveld (EVC) syndrome is a rare genetic abnormality that has been linked to a mutation in the EVC or EVC2 genes. Common atrium (CA) is an uncommon cardiac malformation, and yet it is commonly found in patients with EVC. We performed a retrospective review of the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) between 1982 and 2007. A review of the English-language literature for previously published cases, as well as current genetic research findings, was also performed. Thirty-two pediatric patients with congenital heart disease (CHD) and EVC syndrome were identified in the PCCC database. Twenty-eight (88%) had an endocardial cushion defect, with 15 of these having primary failure of atrial septation resulting in CA. Persistent left superior vena cava (LSVC) and pulmonary venous connection abnormalities were common. The incidence of persistent LSVC and pulmonary venous abnormalities were greater than previously reported for patients with EVC. Our study reviews the reported literature and adds 32 additional cases from the PCCC database. Review of the cardiac phenotype in patients with EVC syndrome reveals a characteristic pattern of atrioventricular canal defects with systemic and pulmonary venous abnormalities. The frequent association of these abnormalities is strongly reminiscent of the cardiac phenotype found in patients with heterotaxy syndromes. Emerging molecular and developmental studies suggest that EVC and EVC2 proteins may be important for cilia function, which is implicated in the pathogenesis of heterotaxy syndromes. It is speculated that coordinate function between the EVC proteins is required for a cilia-dependent cardiac morphogenesis. [ABSTRACT FROM AUTHOR]

A 57-year-old man presented with symptomatic right-to-left shunts decades after surgical closure of an atrial septal defect and partial anomalous pulmonary veins. Two sources of shunting were identified: a baffle leak from the right superior vena cava (RSVC) to the left atrium secondary to complete occlusion of the RSVC-to-right atrium connection and an acquired previously unpublished coronary sinus to the left atrial shunt secondary to inadvertent closure of the coronary sinus. The diagnosis and transcatheter management of these unusual right-to-left shunts are reported. [ABSTRACT FROM AUTHOR]

The newborn can experience two types of differential cyanosis (DC). The common type of DC occurs when oxygen saturation in the right hand is greater than in the foot. The second type of DC, reversed differential cyanosis (RDC), occurs when oxygen saturation is lower in the right hand than in the foot. This phenomenon is observed in transposition of the great arteries (TGA) with patent ductus arteriosis (PDA) and elevated pulmonary vascular resistance or in TGA with PDA and preductal aortic interruption or coarctation. This report describes a case of RDC not previously described involving an infant with supracardiac total anomalous pulmonary venous connection (TAPVC). In supracardiac TAPVC, RDC results from streaming of highly saturated superior vena cava (SVC) blood into the right ventricle, out the main pulmonary artery, through a PDA, and to the descending aorta, with streaming of more desaturated blood from the inferior vena cava (IVC) into the left atrium across the atrial septal defect (ASD)/foramen ovale. Therefore, as part of a neonatal examination to rule out congenital heart disease (CHD), simultaneous pre- and postductal oxygen saturations should be documented. The presence of RDC should initiate immediate full cardiac evaluation for CHD. Supracardiac TAPVC should be included in the differential diagnosis if RDC is observed. [ABSTRACT FROM AUTHOR]

We report the first known case of a retro-aortic innominate vein providing continuity between both the left and right atria in the presence of a left superior vena cava and a normal coronary sinus. This unusual structure created a bidirectional shunt with clinical desaturation at baseline and it complicated the management of a 9-year-old patient while undergoing mechanical ventilation. An embryologic mechanism that explains the development of this complex remains elusive. [ABSTRACT FROM AUTHOR]

Heparin-induced thrombocytopenia (HIT) is a potentially life-threatening, adverse effect of heparin therapy. Patients with this complication require an alternative approach to anticoagulation. Bivalirudin is a direct thrombin inhibitor with an efficacy comparable to that of heparin, a short half-life, and reduced bleeding complications in adults. We present the case of a 2-year-old boy with HIT Type II who underwent recanalization of an occluded superior vena cava and stent placement, utilizing bivalirudin as anticoagulant. [ABSTRACT FROM AUTHOR]

Abstract Transvenous (TV) pacing and defibrillation leads are frequently implanted in children as part of treatment for various congenital and acquired rhythm abnormalities. However, the lead-vascular endothelial interaction is not a benign process and is associated with a risk of progressive venous obstruction. Often, this obstruction requires surgical or interventional relief. The risk of obstruction is related to venous diameters at implant and lead size. Since venous diameters are largely unknown at different ages, the purpose of this study was to correlate innominate vein (iNN) and superior vena cava (SVC) diameters with body dimensions in growing children. [ABSTRACT FROM AUTHOR]

We report an unusual anomaly of the upper body venous drainage in a patient with visceroatrial situs inversus. The patient had a persistent right superior vena cava draining into the left-sided right atrium by way of the coronary sinus, with the absence of the left superior vena cava associated with atrioventricular discordant levocardia, pulmonary atresia, and ventricular septal defect. [ABSTRACT FROM AUTHOR]

Secundum atrial septal defects (ASDs) are routinely closed using transcatheter devices. In patients with left superior vena cava (LSVC) draining to the coronary sinus (CS), the device must not obstruct CS drainage. We report five cases of successful ASD device closure without obstructing flow from this LSVC or dilated CS. [ABSTRACT FROM AUTHOR]

A 2-year-old girl with cor triatriatum with unroofed coronary sinus and persistent left superior vena cava underwent preoperative radiofrequency catheter ablation for atrial tachycardia attack that developed after admission to our hospital. The procedure was not successful. Cryoablation was successfully performed during corrective surgery. Cutting back from the coronary sinus orifice, resection of the fibrous tissue above the mitral valvular orifice, closure of the excised fossa ovalis and coronary sinus orifice with a Gore-Tex patch, and ligation of the persistent left superior vena cava resulted in an excellent hemodynamic outcome. At 2-year follow-up, the patient was free from tachycardia attack. [ABSTRACT FROM AUTHOR]

Since the posterior wall of the right superior vena cava (RSVC) is contiguous with the anterior wall of the right upper pulmonary veins, a localized defect in this common wall may create a cavopulmonary venous confluence without eliminating the normal connection of the same right pulmonary veins with the left atrium (LA). Through this defect, blood of the unroofed right pulmonary veins will drain into the RSVC and right atrium (RA), and blood from the RSVC may shunt into the right pulmonary veins and LA. Hemodynamically, the RSVC will become biatrial. If the RSVC blood flows preferentially into the LA, its right atrial orifice will become stenotic or even atretic. If atretic, the normally positioned RSVC will drain entirely into the LA. In this report, we present the clinical and anatomical findings of two postmortem cases with biatrial drainage of the RSVC. We also document the clinical, echocardiographic, angiocardiographic, and surgical data of a living patient with left atrial drainage of the RSVC and tetralogy of Fallot with pulmonary atresia. The relevant literature and surgical treatment are reviewed, and the morphogenesis of the biatrial and left atrial RSVC is considered. [ABSTRACT FROM AUTHOR]

The absence of a right superior vena cava (SVC) in situs solitus is very rare, and even then it is usually associated with left SVC. There have been few case reports in the literature of isolated bilateral absence of SVC. Bilateral absent SVC can have clinical implication, including pacemaker placements, central venous line placement, monitoring, and occasionally associated anomalies. We describe a case detected on fetal echocardiogram with bilateral absent SVC and structurally normal heart. [ABSTRACT FROM AUTHOR]

An 11-month-old boy with a functionally single ventricle, coronary sinus ostial atresia, and bilateral superior vena cava (SVC) underwent coronary sinus unroofing upon a right bidirectional cavopulmonary shunt. A persistent left SVC left open to guarantee dual coronary venous drainage became dilated with cephalocaudal blood flow reversal and desaturation. The left SVC was surgically ligated on postoperative day 35. [ABSTRACT FROM AUTHOR]

Congenital arteriovenous malformations between the subclavian artery and the superior vena cava (SVC) is a very rare anomaly scantily described in the literature. We describe this anomaly in a 4-month-old infant who presented with congestive heart failure. He was diagnosed to have a right subclavian artery to SVC fistula associated with SVC stenosis .The child successfully underwent device (vascular plug) closure of the fistula and SVC stenting. Six months postprocedure, child is doing well, with complete resolution of symptoms. [ABSTRACT FROM AUTHOR]

We present an infant with unusual anatomy and physiology of a persistent left superior vena cava (LSVC). The adverse physiologic consequences of the LSVC were corrected with percutaneous placement of vascular plugs. [ABSTRACT FROM AUTHOR]

Right superior vena cava (SVC) drainage into the left atrium causing hypoxemia is a very rare congenital malformation. This anomaly can result in complications of chronic cyanosis and paradoxical embolism. It rarely is associated with any other congenital malformation. This is the first case report of right SVC drainage into the left atrium associated with a coronary cameral fistula. During surgical repair, the aneurysmal part of the cameral fistula was noted to be occluding a defect in the atrial septum. Repair of the cameral fistula and rerouting of the SVC via the atrial septal defect were performed. [ABSTRACT FROM AUTHOR]

Absence of the right superior vena cava (SVC) is a rare event occurring in patients without congenital cardiovascular abnormalities. Since absence of the right SVC is usually clinically silent, its diagnosis is mandatory prior to invasive medical or surgical procedures. We report two cases of echocardiographic diagnosis of absence of the right SVC with persistent left SVC and a large coronary sinus in structurally normal heart in a fetus of 20 weeks’ gestation and in a newborn. The diagnosis was confirmed by transthoracic contrast echocardiography with intravenous injection of agitated saline into the right arm. [ABSTRACT FROM AUTHOR]