Maternal and neonatal outcomes in women with congenital heart disease: A nationwide population-based study.

BACKGROUND: We evaluated the outcomes of pregnancy in women with congenital heart disease (CHD) and their offspring in Taiwan. We also investigated how different severity levels may influence the outcomes. METHODS: We used data (2009-2017) from the Birth Certificate Application database in Taiwan, which is linked to the National Health Insurance Research Database and Taiwan Maternal and Child Health Database. We identified 2990 women with CHD who had 4227 births. Based on the CHD subtypes, patients were further divided into "severe CHD" and "simple CHD" groups. RESULTS: Women with CHD have a significant risk of stillbirth. In maternal cardiac events, they had the highest risk of heart failure, followed by arrhythmia. The severity of CHD had a significant effect on the outcomes as well. The neonatal birth event that mothers with CHD have the highest risk of is preterm birth at < 32 weeks of gestation. The prominent difference in neonatal morbidities between mothers with severe and simple CHD is recurrent CHD in the offspring. The offspring of the severe CHD group had a higher risk of severe CHD, whereas those of the simple CHD group had a higher risk of simple CHD. CONCLUSIONS: During pregnancy, the monitoring of heart function and cardiac rhythm could be more intensive in mothers with CHD. In addition to accurately assessing fetal growth and development during antenatal care, mothers with severe CHD should be provided with careful fetal heart structure assessment and genetic testing along with counseling.

Increased Incidence of Kawasaki Disease in Taiwan in Recent Years: A 15 Years Nationwide Population-Based Cohort Study.

Background: Kawasaki disease (KD) is diagnosed in children suffering from fever for more than five days and five clinical characteristic symptoms. The aim of this article was to research the clinical characteristics among KD children in Taiwan in recent years through a population-based cohort study. Materials and Methods: We carried out a nationwide retrospective cohort study by analyzing the data of KD patients (ICD-9-CM code 4461) from Taiwan's National Health Insurance Research Database (NHIRD) during the period of 1996-2011. Results: Among all the insured children in the NHIRD, insurance claims data were reported for 13,260 patients diagnosed with KD, with 8394 (63.30%) subjects being administered IVIG for treatment. Of the patients diagnosed with KD, 94% were under the age of 5 years old, and the majority of cases occurred in May. Furthermore, the incidence of KD more than doubled (28.58-60.08 per 100,000) during this period in Taiwan. Conclusion: We developed a five-based mnemonic device for parents and first-line clinicians to easily use in order to diagnose KD. We also observed an increased incidence of KD in Taiwan during the study period. In addition, we develop a five-based mnemonic device for parents and first-line clinicians in clinical diagnosis of KD can easily remember: Fever> 5 days, 5 clinical criteria, predominantly in children <5 years of age, and peak seasonal clustering in the 5th month, May (April-June) in Taiwan.

Impact of Extracorporeal Membrane Oxygenation on Acute Fulminant Myocarditis-related Hemodynamic Compromise Arrhythmia in Children.

BACKGROUND: Acute fulminant myocarditis (AFM) commonly presents as abrupt cardiogenic shock with or without dysrhythmia. This study evaluated the impact of extracorporeal membrane oxygenation (ECMO) on AFM-related hemodynamic compromise dysrhythmias. We also reported the clinical experience of AFM at our hospital. METHODS: Eighteen children diagnosed with AFM were enrolled. Demographic variables, laboratory data, and clinical courses were reviewed. Thirteen surviving patients with hemodynamic compromise arrhythmia [complete atrioventricular block (CAVB) or ventricular tachycardia (VT)] during hospitalization were divided into Group A (ECMO group; n = 7) and Group B (non-ECMO group; n = 6). RESULTS: The overall survival rate was 78% (14/18). There were no cases of mortality after ECMO was introduced at our hospital. Common symptoms at diagnosis included general malaise (94%), gastrointestinal symptoms (89%), chest pain (56%), shortness of breath (56%), and seizure/syncope (56%). In addition to abnormal cardiac enzyme levels, all patients displayed elevated alanine aminotransferase levels during early disease stages. Electrocardiography at diagnosis revealed dysrhythmia in 15 patients, namely, CAVB in 11 patients (61%) and VT in four patients (22%). During hospitalization, the dysrhythmia shifted from CAVB to VT in 10 patients and from sinus tachycardia to VT in one patient. New episodes of VT were common (overall occurrence rate, 83%). Although myocardial damage and dysfunction were more severe in Group A, the time to rhythm recovery in this group was shorter than that in Group B (median time, 1.7 days vs. 7.35 days, p = 0.045). All surviving patients had normal ventricular function at 6-month follow-up. CONCLUSION: Hemodynamic compromise arrhythmia is common in AFM patients and may cause rapid deterioration. Simply correcting sinus rhythm is not always sufficient because of myocardium instability. Timely use of ECMO can improve the survival rate and shorten the time to recapture sinus rhythm in AFM patients with CAVB or VT.

Common carotid artery intima-media thickness is useful for diagnosis of the acute stage of Kawasaki disease.

BACKGROUND: This study aimed to investigate intima-media thickness (IMT) of the common carotid arteries in children with acute Kawasaki disease (KD). METHODS: Between 2009 and 2011, patients fulfilling the criteria for KD, including a fever lasting >5 days, were prospectively enrolled in this study. Laboratory data, echocardiography, and IMT were measured and compared with matched controls. RESULTS: A total of 70 common carotid IMTs were measured in 35 children. We studied 21 patients aged 3-60 months old with acute KD and 14 febrile patients aged 3-194 months old with acute infection and similar characteristics to those of KD patients. Children with KD had a significantly higher IMT compared with the controls (0.550 ± 0.081 mm vs. 0.483 ± 0.046 mm, P = 0.01). CONCLUSIONS: IMT during the acute stage of KD is increased, suggesting that IMT could be a useful diagnostic tool in the early diagnosis of KD.

Two different approaches to restore renal nitric oxide and prevent hypertension in young spontaneously hypertensive rats: l-citrulline and nitrate.

Nitric oxide (NO) deficiency mediates oxidative stress in the kidney and is involved in the development of hypertension. NO synthesis occurs via 2 pathways: nitric oxide synthase (NOS) dependent and NOS-independent. We tested whether the development of hypertension is prevented by restoration of NO by dietary l-citrulline or nitrate supplementation in young spontaneously hypertensive rats (SHRs). Male SHRs and normotensive Wistar Kyoto control rats (WKYs)s age 4 weeks were assigned to 4 groups: untreated SHRs and WKYs, and SHRs and WKYs that received 0.25% l-citrulline for 8 weeks. In our second series of studies, we replaced l-citrulline with 1 mmol/kg/d sodium nitrate. All rats were sacrificed at age 12 weeks. We found an increase in the blood pressure of SHRs was prevented by dietary supplementation of l-citrulline or nitrate. Both treatments restored NO bioavailability and reduced oxidative stress in SHR kidneys. l-Citrulline therapy reduced levels of l-arginine and asymmetric dimethylarginine (ADMA)-an endogenous inhibitor of NOS-and increased the l-arginine-to-ADMA ratio in SHR kidneys. Nitrate treatment reduced plasma levels of l-arginine and ADMA concurrently in SHRs. Our findings suggest that both NOS-dependent and -independent approaches in the prehypertensive stage toward augmentation of NO can prevent the development of hypertension in young SHRs.

RNA silencing targeting PIN (protein inhibitor of neuronal nitric oxide synthase) attenuates the development of hypertension in young spontaneously hypertensive rats.

Nitric oxide (NO) deficiency contributes to hypertension. We previously showed that neuronal nitric oxide synthase (nNOS) was involved in hypertension and kidney damage in spontaneously hypertensive rats (SHRs). The protein inhibitor of nNOS (PIN) has been reported to inhibit activity of nNOS.Thus, we tested whether increased PIN in the kidney results in hypertension and whether small interfering RNA (siRNA) targeting PIN attenuates hypertension in SHRs. Four-week-old male SHRs were assigned into three groups (n = 6-7/group): SHR; SHR + PIN, SHR that received siRNA targeting PIN; and SHR + NC, SHR treated with random negative control siRNA. Rats were sacrificed at 12 weeks of age. PIN protein expression was inhibited considerably when PIN siRNA was transfected into NRK52E cells (90% siRNA at 1 nM). The increases of BP were attenuated by siRNA targeting PIN in 12-week-old SHRs. Immunostaining of nNOS-α and total nNOS was greater in SHR + PIN group than SHR. Moreover, renal superoxide production and 8-hydroxydeoxyguanosine (8-OHdG) staining were more decreased in the SHR + PIN group than SHRs. We conclude that PIN siRNA reduced PIN expression in vitro and in vivo. PIN siRNA therapy attenuates hypertension in SHRs at 12 weeks of age. Our results suggest that PIN is involved in the development of hypertension.

Predictors for Regression of Large Secundum Atrial Septal Defects Diagnosed in Infancy.

PUPOSE: To determine predictive factors of spontaneous closure or size reduction in large secundum-type atrial septal defects (ASD) diagnosed in infancy prior to catheterization or surgical intervention. METHODS: From June 2003 to October 2009, 59 infants diagnosed with secundum-type ASDs measuring ≥ 8 mm in the first year of life were retrospectively enrolled. We reviewed medical records, as well as electrocardiography and echocardiography findings. Patients were divided into 2 groups according to the last ASD size: group A (n = 23), ASD reduction in size to < 5 mm or spontaneous closure; group B (n = 36), size of ASD remained ≥ 5 mm. RESULTS: The ASDs spontaneously closed in 10 (17%) patients at a median age of 26.0 ± 5.1 months (range, 15-58 months), or decreased to < 5 mm in 13 (22%) (range, 6-27 months) patients. There was a significant difference in age at diagnosis between the 2 groups (p = 0.014). Patients in group A were younger than those in group B at the time of diagnosis. Changes in ASD size (p < 0.001) and body weight percentile (p = 0.01) were also significantly different fromthe 6-month follow-up. ASD diameter of ≥ 10 mm was a negative predictive factor for size reduction (p = 0.017). CONCLUSIONS: Spontaneous closure or size reduction of large ASDs was not uncommon in patients diagnosed during infancy. Patients with initial ASD sizes between 8 and 10 mm who were younger at the time of diagnosis and showed better weight gain were more likely to have favorable outcomes. KEY WORDS: Infancy; Large secundum atrial septal defect; Natural course; Spontaneous closure.

Microsurgical tissue transfers for head and neck reconstruction in patients with alcohol-induced mental disorder.

BACKGROUND: Free tissue transfer in patients with organic mental disorder has always been known to be risky. Herein, the outcomes of free tissue transfers for head and neck reconstruction in those with alcohol-induced mental disorder were analyzed. MATERIALS AND METHODS: We retrospectively reviewed and analyzed data from the past 10 years of 1,364 patients who had undergone microsurgical tissue transfers after head and neck cancer ablation. Among them, 54 patients had been diagnosed with alcohol-induced mental disorders post-operatively. Age ranged from 33 to 71 years. Alcohol-drinking history averaged 17.5 years. Reconstructive procedures included 25 forearm flaps, 13 anterolateral thigh (ALT) flaps, 10 fibula osteocutaneous flaps, and 6 double flaps (fibula+ALT). The outcomes and complications were analyzed. RESULTS: Onset periods ranged from the first to fourth days post-operatively. Duration of alcohol withdrawal or delirium tremens was 3-10 days. All patients gradually stabilized after immediate psychiatric consultation and intensive medical treatment. The flap survival rate in patients with alcohol withdrawal was significantly decreased in comparison with patients not suffering alcohol withdrawal (83% versus 96.4%, P < 0.001). During this critical post-operative period, 28 (52%) patients with alcohol withdrawal syndrome experienced complications; 26 (48%) suffered flap-related complications, and 19 (35.2%) required additional surgery. The analytical parameters revealed that secondary operative procedures and duration of hospitalization differed significantly between the complication and non-complication groups (P < 0.001). CONCLUSION: Higher rates of complications and level of critical care were needed in patients with alcohol-induced mental disorder after head and neck microsurgical reconstructions. Treatment requires a multidisciplinary approach, rapid diagnosis, and intensive medical care.