Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD. METHODS: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019. The left ventricle was assessed in the first 48 hours after birth using two-dimensional, Doppler-derived, six-segment global and segmental longitudinal strain and circumferential strain (CS) and dyssynchrony indices (the SD of time-to-peak strain and a novel global dyssynchrony index [DI], calculated as [peak segmental average - peak global average]/peak segmental average). RESULTS: Neonates with EA/TVD demonstrated reduced combined cardiac index (4.2 ± 1.5 L/min/m2 vs 6.5 ± 2.2 L/min/m2 in control subjects, P < .001), impaired LV CS (-15.4 ± 6.9 vs -26.2 ± 5.8, P < .001), and increased circumferential dyssynchrony (CS DI 0.20 ± 0.16 vs 0.09 ± 0.04 [P = .019]; SD of time-to-peak CS 63 ± 25 vs 40 ± 15 [P = .003]). Transplantation-free survival occurred in 20 of 32 patients (63%) at 6 months. Increased CS DI and absence of pulmonary valve flow (PVF) were most predictive of mortality; CS DI > 0.2 was associated with 25% survival in subjects without PVF, whereas all patients with CS DI < 0.1 survived. CONCLUSIONS: In neonates with EA/TVD and absence of PVF, there is abnormal LV deformation and compromised cardiac output in association with increased dyssynchrony. Increased CS DI is associated with increased risk for mortality in EA/TVD with no forward PVF.

A case of incomplete Kawasaki disease - A 2-month-old infant with 1 day of fever who developed multiple arterial aneurysms.

Kawasaki disease (KD) is a systemic vasculitis and is the most frequent pediatric acquired heart disease in developed countries. The diagnosis of KD is typically made by the: ≧ 5 of 6 principal signs. However, approximately 20% of KD patients present with less than 5 of these diagnostic signs but may be suffering from coronary artery aneurysms and have been diagnosed with incomplete KD. In this case report, we describe a 2-month-old infant who showed just fever without any other signs of KD but was suffered from multiple arterial aneurysms, including coronary, pulmonary, and carotid arteries. Because she did not respond to intravenous immunoglobulin infusion, we placed her on plasma exchange that has successfully brought defervescence without any significant complications. This case may represent the end spectrum of incomplete KD in very young infants.

Simple method to distinguish the type of fetal premature contraction using arterial Doppler time interval measurements.

AIM: The purpose of this study was to establish a simple method to distinguish premature ventricular contractions (PVC) from premature atrial contractions (PAC) using a fetal Doppler ultrasound arterial pulse waveform to measure time intervals between sinus node restarting. METHODS: We retrospectively identified 14 fetuses with premature contraction (8 with PAC, 6 with PVC). We measured two distinct parts of time intervals using an arterial pulsed-wave Doppler: the two consecutive waveforms just before the premature contraction (2-V interval) and two consecutive waveforms including the premature contraction (XV interval) to measure time intervals between sinus node restarting. We then evaluated the time difference between the 2-V and XV intervals in PVC compared to PAC. RESULTS: For PVC, the difference between the 2-V interval and the XV interval was significantly shorter than that for PAC. A cut-off point of 33 ms, where a difference ≤33 ms was clearly shown to be associated with a PVC and a difference more than 33 ms signified a PAC was demonstrated. CONCLUSION: The 2-V and XV interval measurements, used to measure time intervals between sinus node restarting, could easily distinguish PVC from PAC in utero. Therefore, this study could potentially be a feasible and effective method for obstetricians or sonographers to employ usefully.

Intracardiac Echocardiography as a Guide for Transcatheter Closure of Patent Ductus Arteriosus.

BACKGROUND: Transcatheter closure of patent ductus arteriosus (TC-PDA), conventionally guided by aortography, has become the standard treatment of this disease. The purposes of this study were to evaluate whether intracardiac echocardiography (ICE) may be used for measuring PDA size and be used as a guide for TC-PDA. METHODS: This study had 2 phases. In phase 1, we compared the measurements of PDA size: pulmonary artery side diameter (PA-D), length, and aortic side diameter (Ao-D) of PDA, as measured by ICE with those measured by aortography or cardiac computed tomography (AoG/CCT) in 23 patients who underwent TC-PDA. In phase 2, we compared the demographics, fluoroscopic time, contrast volume, and complications of the TC-PDAs between 10 adult patients with ICE guidance and 16 without it. RESULTS: In phase 1, we found great correlation and agreement between ICE and AoG/CCT in PA-D (r = 0.985, bias -0.077 to 0.224), but moderate to poor correlation and agreement in length (r = 0.653, bias -0.491 to 3.065) and Ao-D (r = 0.704, bias 0.738 to 4.732), respectively. Nevertheless, all patients underwent successful TC-PDA with ICE guidance that allowed us to continuously monitor the whole process. In phase 2, TC-PDA required a significantly lower contrast volume with ICE guidance than without it, and there was no significant difference in the remaining variables between the 2 groups. CONCLUSION: ICE is comparable to AoG/CCT in providing accurate PA-D of the PDA and may be a safe alternative to guide TC-PDA as compared to conventional aortography.

Transpulmonary echocardiography to guide stent implantation into coarctation of the aorta.

Although stent implantation into aortic coarctation has been performed solely under fluoroscopy, we successfully applied intracardiac echocardiography (ICE) to guide this procedure in a 13-year-old patient. Placing an intracardiac echocardiographic catheter in the left pulmonary artery facing upward, we readily visualized the precise anatomy of coarctation, measured the pressure gradient, and monitored the stent inflation process. This report suggests a new application of ICE for intervention with structural and vascular diseases other than interatrial septum.

Trans-pulmonary echocardiography as a guide for device closure of patent ductus arteriosus.

OBJECTIVES: The aim of this study was to develop trans-pulmonary echocardiography (TPE) to guide device closure of patent ductus arteriosus (DC-PDA). BACKGROUND: Aortography requires a large amount of contrast yet may give us an inadequate image to evaluate anatomy or residual shunt in patients with large PDA or dilated vessels and is precluded in patients with renal dysfunction. Practically, there is no imaging modality to monitor the entire procedure except for trans-esophageal echocardiography that requires general anesthesia. METHODS: Subjects were seven patients with ages ranged from 6- to 77-years old and body weight > 15 kg. The size of the PDA ranged from 1.8 to 6.3 mm with pulmonary to systemic flow ratios from 1.2 to 2.2. During DC-PDA using Ampaltzer Duct Occluder or coil, an intra-cardiac echocardiographic (ICE) catheter was advanced into pulmonary arteries and standard views were developed to guide DC-PDA. RESULTS: We have developed two standard views; the main pulmonary artery view (MPA view) and the left pulmonary artery view (LPA view). The MPA view provided aortic short axis view equivalent to that seen by trans-thoracic echocardiography in children. The LPA view, obtained by the echo probe in the LPA and turned it up upside down, provided long axis view of the PDA allowing more precise anatomical evaluation. TPE allowed us to monitor the entire procedure and determine residual shunts. CONCLUSIONS: TPE in the MPA and LPA view can be an effective guide for DC-PDA. This report leads to new application of this imaging device.

Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience.

BACKGROUND: Some patients with Kawasaki disease develop giant coronary aneurysms and coronary stenosis, leading to ischemic heart disease. The aim of this study was to determine the long-term prognosis of patients with Kawasaki disease with giant aneurysms. METHODS AND RESULTS: From our institutional database, 76 patients (57 men and 19 women) who developed giant aneurysms after January 1, 1972, were identified. Information on patient demographics, catheter and surgical interventions, and most recent status was collected from medical charts and patients' contacts. From these data, we calculated the survival rate and cumulative coronary intervention rate. The average age at onset was 2.9±2.9 years, and the median observational period was 19 years. During this period, 7 patients died and 1 patient underwent a heart transplantation, resulting in 95%, 88%, and 88% survival rates at 10, 20, and 30 years after the onset of KD, respectively. On the other hand, catheter and surgical coronary interventions (median, 1 intervention; range, 1 to 7 interventions) were performed to alleviate coronary ischemia in 46 patients (61%) at 1 month to 21 years (mode at 1 month) after onset, resulting in 28%, 43%, and 59% cumulative coronary intervention rates at 5, 15, and 25 years after onset, respectively. CONCLUSIONS: The long-term survival of patients with Kawasaki disease complicated by giant coronary aneurysms is moderately good with multiple catheter and surgical interventions. Further research should focus on the prevention of coronary vascular remodeling and on the indications for and effectiveness of percutaneous and surgical coronary interventions.

Increased plasma type B natriuretic peptide in the acute phase of Kawasaki disease.

BACKGROUND: The aim of this study was to identify possible factors associated with type-B natriuretic peptide (BNP) production in the acute phase of Kawasaki disease (KD). METHODS: Subjects were 54 patients with KD (KD group [KDG]) and 18 age-matched controls (control group [CG]). We evaluated left ventricular function using multi-modal echocardiography and determined blood chemistry including BNP, white blood cell count, C-reactive protein (CRP), and interleukin (IL)-6 in the KDG. We compared echocardiographic parameters between the KDG and the CG and determined the correlation between log (BNP) and echocardiographic parameters, white blood cell count, CRP, and IL-6 in the KDG. RESULTS: The KDG showed high BNP (169.6 ± 529.6 pg/ml) despite preserved left ventricular function indicated by no significant difference in left ventricular ejection fraction (72.2 ± 9.2 vs 71.2 ± 7.8 %), z-score of left ventricular diastolic dimension (0.8 ± 1.3 vs 0.9 ± 0.8 SD), and Tei index (0.29 ± 0.09 vs 0.30 ± 0.06) between the KDG and the CG. However, left ventricular ejection fraction (r =-0.44, P= .001) and left ventricular end-diastolic dimension (r = 0.30, P < .05) significantly correlated with log (BNP). On the other hand, the KDG showed high CRP (89.7 ± 55.6 mg/l) and high IL-6 (242.2 ± 243.5 pg/ml), and CRP (r = 0.60, P < 0.0001) and IL-6 (r = 0.78, P < 0.0001) significantly correlated with log (BNP). Multiple stepwise regression analysis identified IL-6 (r = 0.77, P < 0.0001) most significantly correlated with log (BNP). CONCLUSIONS: In acute KD, BNP significantly increases, despite well-preserved global left ventricular function, and inflammation might be associated with this increased BNP.

Microembolic signals measured by transcranial Doppler during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.

PURPOSE: To determine the frequency and factors associated with increase in microembolic signals during transcatheter closure of atrial septal defect using the Amplatzer septal occluder. METHODS: During the procedure in 16 patients, we measured microembolic signals using transcranial Doppler. Procedure time was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; left cardiac angiocardiography; sizing and long sheath placement; device placement and release. We compared numbers of microembolic signals among the five periods and identified factors associated with them. RESULTS: Mean size of septal occluder was 16 millimetres in diameter. Total number of microembolic signals was a median of 31.5, ranging from 3 to 113. Microembolic signals in three periods, left cardiac catheterisation; sizing, and long sheath placement; and device placement and release, were not significantly different from one another, but were significantly higher than those in the remaining two periods, right cardiac catheterisation and left cardiac angiocardiography (median was 9 in left cardiac catheterisation; 6 in sizing and long sheath placement; 6.5 in device placement and release, versus 0 in right cardiac catheterisation and 1 in left cardiac angiocardiography, p less than 0.05, respectively). Importantly, the time for device manipulation positively correlated with total number of microembolic signals (r equals 0.77, p less than 0.001), although fluoroscopic time, age, or size of septal occluder did not. CONCLUSIONS: Transcatheter closure of atrial septal defect using the Amplatzer septal occluder produces microemboli, especially during device placement. To minimise the risk of systemic embolism, we must decrease the time for device manipulation.