Case Report: One heart with two lobes: a rare infantile congenital giant left atrial appendage aneurysm.

Left atrial appendage aneurysm (LAAA) is an extremely rare congenital heart abnormality, with varying degrees of symptoms, ranging from asymptomatic to arrhythmia, thromboembolic event or airway obstruction. Most infantile cases were incidentally found by echocardiography. Contrast-enhanced chest tomography can confirm the diagnosis and inform surgical plan. We describe an asymptomatic young female infant who had a unique extreme cardiomegaly on a chest x-ray and received surgical aneurysmectomy. Her heart was restored to a normal cardiac size after the heart surgery.

Case report: VA-ECMO for fulminant myocarditis in an infant with acute COVID-19.

Fulminant myocarditis in children was rare during the coronavirus disease 2019 pandemic, but it had the potential for high morbidity and mortality. We describe the clinical course of a previously healthy 9-month-old young male infant who rapidly deteriorated into cardiogenic shock due to coronavirus disease 2019-related fulminant myocarditis. He developed severe heart failure and multiple organ dysfunction syndrome that were treated promptly with central venoarterial extracorporeal membrane oxygenation and continuous venovenous hemofiltration. He made a good recovery without significant morbidity.

Novel technique to reduce prolapsed device in atrial septal defect closure.

Objective: Transcatheter closure of atrial septal defect (ASD) has become an alternative treatment to surgical repair. One of the challenges is the prolapse of the left atrial disc during the procedure. Many techniques have been developed to prevent the prolapse but not reduce it. In this study, we present a novel technique, termed push back technique, that help reduce the prolapsed device. Methods: We enrolled 24 patients (8 males, 16 females) between May 2008 and January 2023 who underwent the push back technique during transcatheter closure of ASD in Taichung Veterans General Hospital. We recorded the hemodynamic data, success rate and complications including device embolization/migration, valvular regurgitation, pericardial effusion, and residual shunt. Results: The median age was 6.3 years (1.2-70.5 years) and the median weight was 19.1 kg (7.8-90 kg). Fifteen (62.5%) patients had mild pulmonary hypertension. The median Qp/Qs was 2.54 (1.5-8.8). The median ASD stretched size was 21.2 mm (7.7-35.3 mm). The median device size was 22 mm (8-40 mm). The median fluoroscopy time was 14 min (5-23 min) and median procedure time was 47 min (25-78 min). The push back technique successfully reduced the prolapsed device in 21 (87.5%) patients. There was no complication in all patients. Conclusion: We present a novel push back technique that can successfully reduce the prolapsed device in 87.5% (21/24) patients without complications. It is feasible, safe and effective.

Case report: Transcatheter arterial embolization in a newborn with cervical rapidly involuting congenital hemangioma and Kasabach-Merritt phenomenon.

Congenital hemangiomas (CHs) are rare vascular tumors and do not exhibit progressive postnatal growth. The incidence is less than 3% of all hemangiomas. Most CHs have a favorable prognosis; however, the Kasabach-Merritt phenomenon (KMP) is a rare but life-threatening complication in CHs that requires aggressive treatment. Medical treatments with corticosteroids and interferon have been suggested. Surgical resection can be considered for the treatment of complicated CHs in medically resistant lesions. Vascular embolization could be an alternative method if surgery is not considered feasible. Herein, we report a case of a 9-day-old newborn who underwent arterial embolization for a CH with KMP, combined with sirolimus treatment, and the outcome was favorable. The hemangioma completely regressed by 3 months and rapidly involuting congenital hemangioma (RICH) was diagnosed. Our successful experience with treating RICH associated with KMP revealed that RICH can have potentially serious complications although they usually resolve rapidly after birth without treatment. Surgical resection is considered to be the standard method for the treatment of medically resistant vascular tumors, but it is difficult to perform during the active phase of KMP due to acute bleeding and severe coagulopathy. Arterial embolization is feasible and can be used as an alternative to surgical resection, even in small babies.

Detecting Patent Foramen Ovale after Cryptogenic Stroke - A Single Center Experience in Taiwan.

Background: Cryptogenic strokes related to a patent foramen ovale (PFO) in young patients have been reported. Percutaneous transcatheter occlusion is considered a safe and effective method for achieving PFO closure. Here, we present our experience of PFO closure in a series of young patients diagnosed with cryptogenic stroke. Methods: This was a retrospective study of 62 patients who were younger than 60 years of age and previously diagnosed with cryptogenic stroke. They underwent cardiac catheterization for the diagnosis of PFO and/or device closure. Bubble and echocardiography studies, the patients' characteristics, complications, and recurrence of stroke were recorded. The Risk of Paradoxical Embolism (RoPE) score index was also calculated. Results: Patients with a bubble study grade greater than grade I were likely to have PFO (odds ratio: 22.2 [95% confidence interval: 1.2-412.3]). The mean RoPE score in the PFO closure group was 7.18. Two patients (3.9%) developed atrial fibrillation during the procedure. Ischemic stroke recurred in two patients (3.3%). Conclusions: Combining a bubble study with transcranial Doppler ultrasound or transthoracic echocardiography may increase the validity of PFO diagnosis. Because of the low recurrence rate of stroke and low complication rate, it is important to identify PFO patients with a bubble study grade greater than grade I. Closure of PFO may be a good strategy for preventing recurrent stroke among such patients.

Cutoff Values of Hemodynamic Parameters in Pediatric Refractory Septic Shock.

Background: Refractory septic shock can cause severe morbidities and mortalities in children. Resuscitation based on hemodynamics is important in children with critical illness. Thus, this study aimed to identify the hemodynamics of refractory septic shock associated with poor prognosis at an early stage to allow for timely interventions. Methods: We evaluated children with refractory septic shock admitted to a pediatric intensive care unit (PICU) and monitored their hemodynamics using a pulse index continuous cardiac output (PiCCO) system. The serial cardiac index (CI), systemic vascular resistance index (SVRI), and vasoactive−inotropic score (VIS) were recorded during the first 72 h after PICU admission. Results: Thirty-three children with refractory septic shock were enrolled. The SVRI and VIS were both associated with fatality from septic shock. The non-survivors had lower serial SVRI and higher VIS (both p < 0.05). Based on the area under the ROC curve, the SVRI was the predictor during the early resuscitative stage (first 36 h) in pediatric refractory septic shock. Conclusions: Both SVRI and VIS are predictors of mortality in children with refractory septic shock, and the SVRI is the powerful predictor of mortality in the early resuscitative stage. A low serial SVRI may allow for the early awareness of disease severity and strategies for adjusting vasoactive−inotropic agents to increase the SVRI.

Correlation Between Smoking Paradox and Heart Rhythm Outcomes in Patients With Coronary Artery Disease Receiving Percutaneous Coronary Intervention.

BACKGROUND: The effect of smoking on short-term outcomes among patients with acute coronary syndrome (ACS) undergoing percutaneous coronary intervention (PCI) is controversial. However, little is known about the impact of smoking on long-term outcomes in patients with stable coronary artery disease (CAD) who receive PCI. METHODS: A total of 2,044 patients with stable CAD undergoing PCI were evaluated. They were divided into two groups according to smoking status (current smokers vs. non-smokers). Baseline characteristics, exposed risk factors, angiographic findings, and interventional strategies were assessed to compare the long-term clinical outcomes between groups. Predictors for myocardial infarction (MI), all-cause death, cardiovascular (CV) death, and repeated PCI procedures were also analyzed. RESULTS: Compared with non-smokers, current smokers were younger and mostly male (both P < 0.01). They also had a lower prevalence of chronic kidney disease (CKD) and diabetes (both P < 0.01). Drugs including a P2Y12 receptor inhibitor of platelets (P2Y12 inhibitor), beta-blockers (BB), and statins were used more frequently in current smokers (P < 0.01, P < 0.01, P = 0.04, respectively). Freedom from all-cause death and CV death was lower in the non-smoker group (P < 0.001, P = 0.003, respectively). After adjustment, logistic regression revealed smoking was a major predictor for all-cause death and repeated PCI procedure [hazard ratio(HR): 1.71 and 1.46, respectively]. CONCLUSIONS: Smoker's paradox extends to long-term outcome in patients with stable CAD undergoing PCI, which is partially explained by differences in baseline characteristics. However, smoking strongly predicted all-cause mortality and repeated PCI procedures in patients with stable CAD undergoing PCI.

Isolated partial anomalous origin of a branch pulmonary artery from the descending aorta.

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation using the Amplatzer Vascular Plug. We also set up a diagnostic algorithm to differentiate these diseases from anomalous systemic arterial supply to the pulmonary region. It is possible to make the correct diagnosis using the step-by-step diagnostic algorithm and careful interpretation of chest computed tomography angiography.

Pseudohyperkalemia accompanying actual hyperphosphatemia and hypocalcemia in an adolescent with T-lymphoblastic lymphoma.

Tumor lysis syndrome (TLS) is a life-threatening condition that may occur in patients with lymphoma, leukemia, or cancers with high cellular burdens. Without appropriate treatment, electrolyte imbalances, namely hyperkalemia, hyperphosphatemia, and hypocalcemia, can be fatal in patients with TLS. In pseudohyperkalemia, concurrent hyperphosphatemia and hypocalcemia can render devising a treatment strategy challenging. We report an adolescent with T-lymphoblastic lymphoma who presented with pseudohyperkalemia but actual hyperphosphatemia and hypocalcemia, to highlight the importance of accurate clinical interpretations of laboratory data in patients with TLS.

Catecholamine-Induced Secondary Takotsubo Syndrome in Children With Severe Enterovirus 71 Infection and Acute Heart Failure: A 20-year Experience of a Single Institute.

Background: Acute heart failure (AHF) is the major cause of death in children with severe enterovirus 71 (EV71) infection. This study aimed to report our clinical experience with EV71-related AHF, as well as to discuss its pathogenesis and relationship to Takotsubo syndrome (TTS). Methods: A total 27 children with EV71-related AHF between 1998 and 2018 were studied. The TTS diagnosis was based on the International Takotsubo Diagnostic Criteria. Results: Acute heart failure-related early death occurred in 10 (37%) of the patients. Sinus tachycardia, systemic hypertension, and pulmonary edema in 100, 85, and 81% of the patients, respectively, preceded AHF. Cardiac biomarkers were significantly increased in most patients. The main echocardiographic findings included transient and reversible left ventricular (LV) regional wall motion abnormality (RWMA) with apical ballooning. High concentrations of catecholamines either preceded or coexisted with AHF. Myocardial pathology revealed no evidence of myocarditis, which was consistent with catecholamine-induced cardiotoxic damage. Patients with EV71-related AHF who had received close monitoring of their cardiac function, along with early intervention involving extracorporeal life support (ECLS), had a higher survival rate (82 vs. 30%, p = 0.013) and better neurological outcomes (59 vs. 0%, p = 0.003). Conclusion: EV 71-related AHF was preceded by brain stem encephalitis-related hypercatecholaminemia, which resulted in a high mortality rate. Careful monitoring is merited so that any life-threatening cardiogenic shock may be appropriately treated. In view of the similarities in their clinical manifestations, natural course direction, pathological findings, and possible mechanisms, TTS and EV71-related AHF may represent the same syndrome. Therefore, we suggest that EV71-related AHF could constitute a direct causal link to catecholamine-induced secondary TTS.

Ultrasonographic measurements of femoral vessel diameter in neonates weighing less than 2.5 kg.

BACKGROUND: Cannulation in low birth weight (LBW) neonates using larger sheaths could increase the risk of vascular injury. This study investigated the relationship between body weight (BW) and diameter of femoral vessels in LBW neonates and whether BW can be used to predict femoral vessel diameter. METHODS: The cohort included 100 neonates weighing < 2.5 kg (.57-2.42 kg) with a gestational age of 24-39 weeks. Vascular ultrasonography was used to measure diameters of the bilateral femoral arteries (FA) and veins (FV). The cohort was divided into four groups according to weight: group-A, 2-2.49 kg (n = 28); group-B, 1.5-1.99 kg (n = 38); group-C, 1-1.49 kg (n = 21); and group-D, < 1 kg (n = 13); or according to BSA: group-A, BSA > .16 m2 (n = 25); group-B, .13-.16 m2 (n = 40); group-C, .1-.13 m2 (n = 22); and group-D, < .1 m2 (n = 13). RESULTS: The median vessel diameters (mm) in groups A-D according to weight were FA, 1.96, 1.86, 1.78, and 1.53, and FV, 2.30, 2.28, 2.13, and 1.87, respectively. The median vessel diameters (mm) in groups A-D according to BSA were FA, 1.96, 1.86, 1.76, and 1.53, and FV, 2.30, 2.28, 2.05, and 1.87, respectively. There were positive correlations between BW and femoral vessel diameter (correlation coefficient: .56 and .55 between BW and FA and FV, respectively) (p < 0.001), and between BSA and femoral vessel diameter (correlation coefficient: .56 and .55 between BSA and FA and FV, respectively) (p < 0.001). CONCLUSIONS: BW is a predictor of femoral vessel diameter in LBW newborns. This finding may help to avoid using larger sheath in smaller vessels.

Transcatheter closure of post-procedural right ventricular pseudoaneurysms in a neonate with pulmonary atresia.

Post-procedural right ventricular pseudoaneurysm is a rare but life-threatening complication of interventional catheterisation. We describe a 3-day-old newborn who underwent transcatheter intervention for pulmonary atresia with a complication of right ventricular pseudoaneurysms, and transcatheter embolisation of the pseudoaneurysms was performed at 3-week-old. It is the first described case that receives transcatheter closure of right ventricular pseudoaneurysms in a newborn with a favourable outcome.

Urine catecholamines in children with severe Enterovirus A71 infection: comparison with paediatric septic shock.

Purpose: Hypercatecholaminemia-related heart failure has been proposed as the main cause of enterovirus A71-related (EV-A71) early mortality. The purpose of this study was to measure urine catecholamine concentrations in severe EV-A71-infected children. Methods: A total of 35 children, aged 2.5 ± 2.1 years, were divided into three groups. Group I: 15 septic shock patients, group II: 17 EV-A71-stage-2 patients, and group III: 3 EV-A71-stage-4 patients. The laboratory results, cardiac biomarkers and urine catecholamine concentrations were statistically analysed. Results: Group I had the highest C-reactive protein (CRP) levels and group II had the lowest B-type natriuretic peptide (BNP) and its N-terminal prohormone among the groups (p = 0.039, <0.01 and <0.01, respectively). Group III patients had significantly higher urine catecholamine and troponin-I values among the groups. If urine epinephrine (Epi) >134 ug/gCr, norepinephrine (NE) >176 ug/gCr and vanillylmandelic acid (VMA) >11.7 mg/gCr were used as the cutoff points to differentiate groups II and III, the sensitivities and specificity were all 100%. Conclusions: The significantly elevated urine catecholamine concentrations in EV-A71-stage-4 patients support the hypothesis that hypercatecholaminemia-related heart failure is involved in severe EV-A71 infection. Urine catecholamines could be used as reliable biomarkers for differentiation of severe EV-A71 infection with or without heart failure and septic shock.

Mid-term follow-up study of neonatal isolated aberrant right subclavian artery.

BACKGROUND: Aberrant right subclavian artery is the most common congenital aortic arch anomaly. There are a few reports concerning the clinical manifestations and follow-up of this CHD detected by echocardiographic screening. METHODS: A total of 1737 full-term neonates, with a male-to-female ratio of 900:837, received echocardiographic screening. Neonates with or without isolated aberrant right subclavian artery were studied during infancy. RESULTS: Among the 1737 cases, a total of 15 (0.86%) female-predominant neonates, with a male-to-female ratio of 6:9, had isolated aberrant right subclavian artery. They were compared with 20 age- and gender-matched normal neonates. There were no significant differences in maternal age, gestational age, or para gravity between the two groups, except for birth size (birth length- and weight-for-age percentiles, p = 0.006 and 0.045, respectively), which was smaller in the aberrant right subclavian artery group. Although there were no significant differences in developmental history, gastrointestinal, or respiratory symptoms, neonates with aberrant right subclavian artery had a higher incidence of mild developmental delay and feeding difficulty than normal infants (21 versus 0%, p = 0.061; 36 versus 20%, p = 0.264, respectively). The growth rates of body length and weight during infancy were not significantly different between the two groups. CONCLUSIONS: Echocardiography can be applied as a first-line investigation in the diagnostic work-up for aberrant right subclavian artery. Neonates with aberrant right subclavian artery had a smaller size at birth, although growth rates were not significantly different from those of normal infants. This study does not support an active surgical policy for asymptomatic infants with isolated aberrant right subclavian artery. A larger study and longer follow-up of affected infants is recommended.

Vascular Plugging for the Enlarging Pulmonary Arteriovenous Malformation in a Patient With Idiopathic Pulmonary Artery Hypertension.

The coexistence of idiopathic pulmonary artery hypertension with pulmonary arteriovenous malformation (PAVM) is challenging because although the PAVM causes hypoxia and polycythemia with potential thrombotic complications, closing the PAVM increases pulmonary artery pressure. We report on a lady with PAVM and idiopathic pulmonary artery hypertension who, within 2 years of diagnosis, had an ischemic stroke, PAVM enlargement from 20 × 20 × 30 mm to 30x × 40 × 40 mm and oximetry decrease to 90%. Transcatheter occlusion of PAVM with a vascular plug was successful. A year later, she had no flow via the PAVM and systemic oximetry improved to 97%, but pulmonary vascular resistance increased to 1.5-fold of baseline.

Initial experience of transcatheter closure of subarterial VSD with the Amplatzer duct occluder.

BACKGROUND: The traditional treatment of subarterial ventricular septal defect (VSD) is open heart surgery. This study aimed to evaluate the feasibility, safety and outcome of transcatheter closure with the Amplatzer duct occluder (ADO). METHODS: Between March 2012 and June 2015, a total of 16 patients (8 male and 8 female) with subarterial VSD who underwent transcatheter closure with the ADO were enrolled retrospectively. Their age ranged from 3.0 to 65.6 years, with the median of 35.6 years; their body weights ranged from 14 to 92 kg with the median of 60 kg. All patients had prolapse of the right coronary cusp without subaortic rim. Mild aortic regurgitation was noted in 11 (69%) patients. RESULTS: Left ventriculogram showed VSD size ranged from 1.3 to 9.3 mm with the median of 3.5 mm. The device was successfully implanted in 88% (14/16) of the patients. Although one patient had mild skin allergy to contrast medium, no other complication was noted. Complete closure rate was 64%, 86%, 86% and 86% at 1-day, 1-month, 6-month and 12-month follow-up, respectively. Two patients developed new or worsening aortic regurgitation during follow-up. CONCLUSION: Transcatheter closure of subarterial VSD with ADO is technically feasible and safe in patients older than 7 years of age. However, development or worsening of aortic regurgitation requires long-term follow-up.

Transcatheter device closure of postmyocardial infarction ventricular septal defect.

BACKGROUND: Transcatheter device closure of postmyocardial infarction ventricular septal defect (PMIVSD) is less invasive than surgical repair. However, its feasibility, timing, outcome, and prognostic factors remain unclear. METHODS: This was a multicenter, retrospective cohort study. Between February 2012 and July 2015, a total of 10 (8 male and 2 female) patients with PMIVSD undergoing attempted device closure were enrolled retrospectively. The procedures were performed under general anesthesia with fluoroscopic and transesophageal echocardiographic guidance. RESULTS: The patients enrolled in the study were in the age range 50-85 years (median age of 76.5 years). The interval from infarction to device closure ranged from 6-147 days, with the median of 12 days. A total of 13 devices were implanted in 10 patients. There were five Amplatzer muscular ventricular septal defect occluders, four Amplatzer septal occluders, three Amplatzer PMIVSD occluders and one Amplatzer vascular plug II. Complications included transient ventricular tachycardia in three patients, device embolization in one patient, and tracheal bleeding in one patient. No procedure-related death, stroke, or cardiac tamponade was noted. During follow-up, two patients died of heart failure and two patients died of sepsis. Overall, subjects with age ≥ 80 years, systolic blood pressure ≤ 90 mmHg, and procedure time ≥180 minutes were significant predictor factors for mortality. All patients with the interval of infarction to device closure >12 days survived. CONCLUSION: Our findings indicate that transcatheter device closure of PMIVSD is technically feasible, safe, and effective to reduce the shunt. The crucial prognostic factors were ascertained to be age ≥ 80 years, systolic blood pressure ≤ 90 mmHg, and procedure time ≥180 minutes.