Stridor as a Harbinger of Congenital Cardiovascular Anomaly.

BACKGROUND: There is an intimate spatial relationship between the cardiovascular and airway structures. Central airway compression related to congenital cardiovascular anomalies should be considered in neonates, infants, and young children presenting with stridor. METHODS: From July 31, 1990 to December 31, 2018, 24 pediatric patients, including 18 males and 6 females, aged 1 day to 11.3 years old, presenting with stridor and/or lip cyanosis were enrolled in this study. At presentation, none of the patients had a known history of congenital heart disease. Patients with congenital bronchopulmonary vascular/foregut malformations, congenital pulmonary venolobar syndrome, congenital pulmonary malinosculations, Eisenmenger syndrome, secondary pulmonary hypertension, and idiopathic pulmonary arterial hypertension were excluded from this study. Available profiles of 24 patients were reviewed and the underlying congenital cardiovascular anomalies contributing to the clinical manifestation of stridor were analyzed, including chart recordings, chest radiograms, echocardiograms, computerized tomography, electrocardiograms, esophagograms, cardiac catheterization, magnetic resonance imaging, and bronchography. RESULTS: Stridor was an early sign of congenital cardiovascular anomalies, including double aortic arch, right aortic arch (RAA) with Kommerell diverticulum, mirror-image right aortic arch with aortic diverticulum, anomalous right innominate artery, left pulmonary artery sling, RAA with tetralogy of Fallot and persistent fifth aortic arch, a vertical patent ductus arteriosus from a transverse left aortic arch, and absent pulmonary valve syndrome. Notably, chest radiography provided the first clue of RAA in 18 of the 24 patients. CONCLUSIONS: Stridor can be a harbinger of congenital cardiovascular anomalies causing central airway compression in pediatric patients.

KMUP-1 Ameliorates Ischemia-Induced Cardiomyocyte Apoptosis through the NO⁻cGMP⁻MAPK Signaling Pathways.

To test whether KMUP-1 (7-[2-[4-(2-chlorophenyl) piperazinyl]ethyl]-1,3-dimethylxanthine) prevents myocardial ischemia-induced apoptosis, we examined KMUP-1-treated H9c2 cells culture. Recent attention has focused on the activation of nitric oxide (NO)-guanosine 3', 5'cyclic monophosphate (cGMP)-protein kinase G (PKG) signaling pathway triggered by mitogen-activated protein kinase (MAPK) family, including extracellular-signal regulated kinase 1/2 (ERK1/2), c-Jun N-terminal kinase (JNK), and p38 in the mechanism of cardiac protection during ischemia-induced cell-death. We propose that KMUP-1 inhibits ischemia-induced apoptosis in H9c2 cells culture through these pathways. Cell viability was assessed using MTT (3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide) assay and apoptotic evaluation was conducted using DNA ladder assay and Hoechst 33342 staining. The level of intracellular calcium was detected using - Fura2-acetoxymethyl (Fura2-AM) staining, and mitochondrial calcium with Rhod 2-acetoxymethyl (Rhod 2-AM) staining under fluorescence microscopic observation. The expression of endothelium NO synthase (eNOS), inducible NO synthase (iNOS), soluble guanylate cyclase α1 (sGCα1), PKG, Bcl-2/Bax ratio, ERK1/2, p38, and JNK proteins were measured by Western blotting assay. KMUP-1 pretreatment improved cell viability and inhibited ischemia-induced apoptosis of H9c2 cells. Calcium overload both in the intracellular and mitochondrial sites was attenuated by KMUP-1 pretreatment. Moreover, KMUP-1 reduced intracellular reactive oxygen species (ROS), increased plasma NOx (nitrite and nitrate) level, and the expression of eNOS. Otherwise, the iNOS expression was downregulated. KMUP-1 pretreatment upregulated the expression of sGCα1 and PKG protein. The ratio of Bcl-2/Bax expression was increased by the elevated level of Bcl2 and decreased level of Bax. In comparison with the ischemia group, KMUP-1 pretreatment groups reduced the expression of phosphorylated extracellular signal-regulated kinases ERK1/2, p-p38, and p-JNK as well. Therefore, KMUP-1 inhibits myocardial ischemia-induced apoptosis by restoration of cellular calcium influx through the mechanism of NO-cGMP-MAPK pathways.

Revascularization of Concurrent Renal and Cerebral Artery Stenosis in a 14-Year-Old Girl with Takayasu Arteritis and Moyamoya Syndrome.

Concurrent involvement of bilateral renal and cerebral arteries, usually incurred as stenosis, is rare in childhood-onset Takayasu arteritis (c-TA). We report the case of a 14-year-old girl, with c-TA, presenting with transient ischemic attack after endovascular revascularization for renal artery stenosis and cerebrovascular stroke after surgical revascularization for cerebral artery stenosis associated with childhood-onset moyamoya syndrome. We deem that decrease of blood pressure by endovascular revascularization and improvement of cerebral perfusion by surgical revascularization may have jeopardized the cerebral deep watershed zone to cerebral ischemia followed by cerebral hyperperfusion syndrome and caused transient ischemic attack and cerebrovascular stroke in our patient. Revascularization could be a double-edge sword for c-TA patients presenting with concomitant renal artery stenosis and cerebral artery stenosis, and should be performed with caution. Quantitative analysis of cerebral blood flow by brain magnetic resonance imaging and angiography should be performed within 48 hours after surgical revascularization in c-TA.

The Concept of the Arch Window in the Spiral Switch of the Great Arteries.

When the arterial switch operation includes the Lecompte maneuver, the arterial trunks are reconnected in parallel, rather than the spiral fashion observed in the normal heart. Thus, although the ventriculo-arterial connections are hemodynamically corrected, the anatomic arrangement cannot be considered normal. We hypothesized that, if feasible, it would be advantageous to restore a spiral configuration for the arterial trunks. In 58 patients, we reconstructed the arterial trunks such that, postoperatively, the pulmonary channel spirals round the aorta, passing to either the right or the left, and branches posteriorly. We compared the outcomes with those in 95 patients undergoing a standard non-spiraling operation over the same period. Average follow-up was 8.2 ± 4.5 years. The estimated 10-year survival was similar in the cohorts, at 94.7 % for those with spiraling trunks, as compared to 90.4 % for those with parallel outflow tracts. Reoperation-free survival at 10 years was not significantly different (87.6 vs. 90.5 %). Supravalvar pulmonary stenosis, aortic neo-coarctation, or left bronchial stenosis, however, was encountered in one-eighth of those undergoing a standard operation. None of these complications occurred in those patients who, postoperatively, had spiraling outflow tracts (P = 0.002). Reconstruction of spiraling trunks after the arterial switch has, thus far, avoided the complications of supravalvar pulmonary stenosis, neo-aortic kinking, or bronchial stenosis. The spiraling arrangement prevents compression of the pulmonary vessels and bronchial tree by the aorta, since it provides a wide window in the new aortic arch.

Angioplasty for recurrent renal artery stenosis in flare-up of Hashimoto's thyroiditis.

Takayasu arteritis is the most common disease seen in children presenting with renovascular hypertension (RVH) in Asia, and can manifest anatomically as renal artery stenosis (RAS). We report the case of a 16-year-old girl presenting with RVH due to recurrent and novel RAS in Hashimoto's thyroiditis (HT) flare-up. After treatment with thyroxin and percutaneous transluminal renal angioplasty (PTRA), she was free of hypothyroidism and systemic hypertension. RVH due to recurrent and novel RAS in HT flare-up has not previously been reported in the English-language literature. PTRA is the procedure of choice, providing there is no renal artery dissection or aneurysm.

Wheezing as a sign of cor triatriatum sinister culminating in multiple organ failure.

Cardiac asthma or cardiac wheezing (CW) refers to a syndrome of dyspnea and wheezing that mimicks asthma clinically. Reported herein is the case of a 2-month-old boy who presented with refractory wheezing as a sign of cor triatriatum sinister (CTS) that culminated in overwhelming multiple organ failure in a short time. On the day of admission, oxygen saturation (SpO2 ) was <80%. Heart rate was 198 beats/min and respiratory rate 58 breaths/min. Chest radiogram showed pulmonary edema. Electrocardiogram showed right atrial enlargement and right ventricular hypertrophy. N-terminal pro-brain natriuretic peptide (NTproBNP) was very high at >20,000 pg/mL. Two-dimensional echocardiography with Doppler showed CTS, which was complicated with severe pulmonary arterial hypertension due to flagrant pulmonary venous obstruction. Cardiac surgery was undertaken, after which pulmonary edema subsided, SpO2 increased to ≥96%, and NTproBNP dropped to normal. He was discharged 11 days later, and was free of cardiac, pulmonary, renal, and neurological sequelae at 24 month follow up.

Cutting balloon angioplasty for in-stent restenosis of the aortic coarctation in a young boy presenting with systemic hypertension of the upper extremities.

An 8.25-year-old boy was incidentally found to have systemic hypertension of the upper extremities. Blood pressures of the upper extremities were 142-150/86-98 mmHg, and those of the lower extremities 110-116/60-66 mmHg. Doppler echocardiography showed in-stent restenosis of the aortic coarctation. Traditional high-pressure balloon angioplasty failed to dilate this inveterate in-stent restenosis. Instead, a cutting balloon angioplasty was performed. The lumen was dilated from 4.80 mm to 7.89 mm. The pressure gradient dropped from 32 mmHg to 9 mmHg. Blood pressures of the upper extremities were 112-116/76-78 mmHg, and those of the lower extremities 100-104/70-72 mmHg. This paper highlights that a cutting balloon angioplasty can serve as a juste milieu to relieve in-stent restenosis of the aortic coarctation when traditional high-pressure balloon angioplasty is debatable.

Two-stage endovascular balloon angioplasty and stent implantation for supravalvular pulmonary stenosis in a 33-month-old boy incurring congestive heart failure following arterial switch operation.

We report a 33-month-old boy, who had undergone arterial switch operation (ASO) for simple transposition of the great arteries (TGA) since birth, presenting with exercise intolerance with dyspnoea, tachypnoea, tachycardia, cardiomegaly and hepatomegaly. Doppler echocardiography showed severe tricuspid regurgitation and dilatation of the right atrium. At 28 months, cardiac catheterization tracked down the culprit to be right ventricular outflow tract obstruction (RVOTO) manifested at the mid-portion of the main pulmonary artery (MPA) as supravalvular pulmonary stenosis (SVPS) and measuring 2.98 mm in diameter. There was a pressure gradient of 76 mmHg between the right ventricle and the distal portion of the MPA. The lumen was initially dilated to 6.79 mm by balloon angioplasty (BA), after which the pressure gradient dropped to 51 mmHg. However, cardiomegaly and hepatomegaly lingered for 5 months. Stent implantation (SI) was performed at 33 months, after which the lumen was dilated to 9.99 mm and the pressure gradient dropped to 20 mmHg. Liver span decreased from 10 cm to 6 cm.There was a recrudescence of physical activity. Echocardiography showed regression of tricuspid regurgitation and dilatation of the right atrium at the 12-month follow-up.

Clinical features, acute complications, and outcome of Salmonella meningitis in children under one year of age in Taiwan.

BACKGROUND: Salmonella meningitis remains a threat to children below two years of age in both developing and developed countries. However, information on such infections has not been well characterized. We analyzed data related to twelve years of experience in order to clarify the comprehensive features of Salmonella meningitis in our patients, including admission characteristics, acute complications, and long-term outcome. METHODS: The records of patients with spontaneous Salmonella meningitis from 1982 to 1994 were retrospectively reviewed. The long-term outcome was prospectively determined for survivors at school age by the developmental milestones reported by their parents and detailed neurological evaluation along with intelligence, hearing, visual, speech and language assessments. RESULTS: Of the twenty-four patients, seizures were noted in fifteen (63%) before admission and thirteen (54%) during hospitalization. Acute complications mainly included hydrocephalus (50%), subdural collection (42%), cerebral infarction (33%), ventriculitis (25%), empyema (13%), intracranial abscess (8%), and cranial nerve palsy (8%). Three patients (13%) died during the acute phase of Salmonella meningitis. The twenty-one survivors, on whom we followed up at school age, have sequelae consisting of language disorder (52%), motor disability (48%), intelligence quotient < 80 (43%), epilepsy (33%), sensorineural hearing loss (17%), visual deficits (10%), abducens nerve palsy (5%), microcephaly (5%), and hydrocephalus (5%). Overall, good outcome was noted in six (28.6%) of twenty-one survivors, mild sequelae in three (14.2%), moderate in six (28.6%), and severe in six (28.6%). CONCLUSION: Salmonella meningitis in neonates and infants had a wide spectrum of morbidity and acute complications, leading to a complicated hospital course and subsequently a high prevalence of permanent adverse outcome. Thus, early recognition of acute complications of Salmonella meningitis and a follow-up plan for early developmental assessment of survivors are vital.

Systemic hypertension followed by insidious stroke in a 12-year-old boy with childhood neurofibromatosis type 1 presenting with renal and cerebral artery vasculopathy.

Lee ML, Chang TM, Yang RC, Yang AD, Chen M. Systemic hypertension followed by insidious stroke in a 12-year-old boy with childhood neurofibromatosis type 1 presenting with renal and cerebral artery vasculopathy. Turk J Pediatr 2019; 61: 629-634. Neurofibromatosis type 1 (NF1)-associated vasculopathy is usually diagnosed decades after the clinical diagnosis of NF1. Childhood NF1-associated renal artery vasculopathy or moyamoya-like brain vasculopathy could be clinically silent for a long time. We report a 12-year-old boy who had systemic hypertension found incidentally at a routine check-up. Physical examination showed caféau- lait spots and strong radial pulses. Abdominal computerized tomography angiography showed severe right ostial renal artery stenosis. Genomic study showed a heterozygous mutation c.5902C > T (p.R1968*) and two heterozygous single nucleotide polymorphisms (NCBI: SNP rs18011052 and rs2905876) of NF1 gene. After endovascular revascularization for renovascular hypertension caused by renal artery stenosis, including percutaneous transluminal renal angioplasty and stent implantation, blood pressure dropped effectively from 205/143 mmHg to 130/90 mmHg. Supine renin level dropped from 87.2 pg/ mL to 47.9 pg/mL. Unfortunately, right hemiplegia, transient visual loss with blind spots (scotomas), and clumsiness of extremities emerged insidiously 3.5 months later. Brain magnetic resonance imaging and magnetic resonance angiography showed ischemic infarction involving the watershed area of the anterior and middle cerebral arteries, indicating presence of moyamoya-like brain vasculopathy. A dilemma is that a significant decrease of blood pressure after endovascular revascularization for renal artery stenosis may have potentially unmask the moyamoya-like brain vasculopathy in this patient. Vasculopathy could be heralding childhood NF1 in the young patients without full-fledged clinical features. Endovascular revascularization for renal artery stenosis could be a double-edge sword in childhood NF1 presenting with concomitant renal and cerebral artery vasculopathy.

Major determinants and long-term outcomes of successful balloon dilatation for the pediatric patients with isolated native valvular pulmonary stenosis: a 10-year institutional experience.

PURPOSE: We report herein major determinants and long-term outcomes of balloon dilatation (BD) for 27 pediatric patients with isolated native valvular pulmonary stenosis (VPS). MATERIALS AND METHODS: From May 1997 to May 2003, 27 pediatric patients with VPS (pressure gradients >or= 40 mmHg) were enrolled in this retrospective study. Single-balloon maneuver was applied in 26 patients, and double-balloon maneuver in 1. After BD, the pressure gradients were documented simultaneously by pullback maneuver by cardiac catheterization and echocardiography within 24 hours, at 1-month, 3-month, 1-year, and 4-to-10-year follow-ups. RESULTS: Before BD, the echocardiographic gradients ranged from 40 to 101 mmHg (61+/-19, 55), and from 40 to 144 mmHg (69+/-32, 60) by pressure recordings. After BD, the gradients ranged from 12 to 70 mmHg (29+/-13, 27) by pressure recording (p<0.001), and from 11 to 64 mmHg (27+/-12, 26) by echocardiography within 24 hrs (p<0.001). The ratios of the systolic pressure of the right ventricle to those of the left ventricle were 55 to 157% (89+/-28, 79%) before BD, and 30 to 79% (47 +/- 13, 42%) after BD (p<0.001). Follow-up (7.7+/-5.7, 4.5 years) echocardiographic gradients ranged from 11 to 61 mmHg (25+/-11, 24). Two patients did not have immediate success owing to infundibular spasm. Improved right ventricular compliance could be accounted for the ultimate success in these 2 patients. The ultimate successful rate was 100%. CONCLUSION: BD can achieve excellent long-term outcomes in the pediatric patients with isolated native VPS.

A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

201Tl Myocardial Perfusion Imaging in a Case of Double-Inlet Left Ventricle: Correlate Anatomic Information of SPECT With MRI.

Double-inlet left ventricle (DILV) is a congenital heart disease that only a single left ventricle and a rudimentary right ventricle are developed. Because of lack of anatomic landmark, interpretation of Tl myocardial perfusion imaging in DILV is essentially challenging for unusual anomaly. We report the case of a 44-year-old woman with DILV who presented exertional dyspnea and tachycardia and underwent Tl SPECT. The anatomic characteristics of cardiac Tl SPECT are correlated with MRI. To obtain accurate interpretation in DILV, it is absolutely necessary to realize the anatomy relationship between perfusion imaging and anatomic imaging such as CT or MRI.

Diagnosis of the double aortic arch and its differentiation from the conotruncal malformations.

PURPOSE: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases. MATERIALS AND METHODS: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed. RESULTS: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnostic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology. CONCLUSION: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein.

PURPOSE: Four pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins. MATERIALS AND METHODS: From January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies. RESULTS: Clinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired. CONCLUSION: The clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Tibial agenesis-ectrodactyly syndrome associated with novel cardiovascular and bronchopulmonary malformations.

We report a 9-month-old female infant presenting, on the 15th day of life, left tibial agenesis with ectrodactyly, right talipes equinovarus, syndactyly of the second and the third toes of the right foot, an incomplete split right foot, left fibular hypoplasia, bronchial stenosis/hypoplasia, and coarctation of the aortic arch. To the best of our knowledge, coarctation of the aortic arch and bronchial stenosis/hypoplasia have never been reported in the tibial agenesis-ectrodactyly syndrome.

Influence of apical position on the left ventricular outflow tract obstruction in congenitally corrected transposition.

BACKGROUND: The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval ipsilaterality, which may influence the outcome of the double switch operation (DSO). The goal of this study was to determine if the LVOT is compressed by the right ventricle in this setting. METHODS: A total of 103 patients with CCT were divided into four groups according to ventricular looping and apical position, including Group A (D-loop and levocardia), Group B (L-loop and dextrocardia), Group C (D-loop and dextrocardia), and Group D (L-loop and levocardia). Computed tomography was used to define left-right laterality and ventro-dorsal relationship of the LVOT. RESULTS: Apicocaval ipsilaterality was found in 57 patients (Group A, n=25; Group B, n=32), in whom the right ventricle was found to wrap around the LVOT. Among them, 49 (86%) had LVOT obstruction. In 46 patients without apicocaval ipsilaterality (Group C, n=10; Group D, n=36), 31 had LVOT obstruction (67.4%). LVOT obstruction was more prone to occur in patients with apicocaval ipsilaterality compared with those without (p=0.025), and was more significant in the situs solitus (p=0.058) than in situs inversus (p=0.547). CONCLUSIONS: LVOT obstruction was prone to occur in CCT patients with situs solitus and apicocaval ipsilaterality (Group B). The ventricular outflow patency was influenced by apical position, which should be considered to avoid a posterior ventricular outflow tract from compression after DSO.