Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease.

BACKGROUND: In 2017, the AHA published revised guidelines for the diagnosis of Kawasaki disease (KD). In the absence of compelling data supporting or refuting the utility of lack of tapering (LT) and perivascular brightness (PB), expert panel consensus removed LT and PB from consideration. We hypothesize that LT and PB are unreliable, subjective findings, non-specific to KD, which can be seen in systemic febrile illnesses without KD and in normal controls. METHODS: We performed a single-center retrospective study from 1/2008 to 12/2016. De-identified coronary artery (CA) echocardiographic clips from patients 0-10 years old were interpreted blindly by six pediatric cardiologists. Subjects were grouped as follows: (1) healthy: afebrile with benign murmur, (2) KD: IVIG treatment, 4-5 clinical criteria at presentation, (3) incomplete KD (iKD): IVIG, 1-3 clinical criteria, (4) Febrile: ≥3 days of fever, no IVIG, KD not suspected. The presence or absence of LT and PB was recorded. Inter-rater and intra-rater reliabilities were analyzed using intra-class correlation coefficient, Fleiss' Kappa and Cohen's Kappa coefficients. RESULTS: We interpreted 117 echocardiograms from healthy (27), KD (30), iKD (32), and febrile (28) subjects. Analysis showed moderate agreement in CA z score measurements. LT and PB were observed by most readers in control groups. LT exhibited fair inter-reader agreement (reliability coefficient 0.36) and PB slight inter-reader agreement (reliability coefficient 0.13). Intra-rater reliability was inconsistent for both parameters. CONCLUSIONS: LT and PB are subjective, poorly reproducible features that can be seen in febrile patients without KD and in healthy children.

Photoplethysmographic assessment of pulse transit time correlates with echocardiographic measurement of stroke volume in preterm infants with patent ductus arteriosus.

OBJECTIVE: We aimed to correlate photoplethysmographic parameters with stroke volume in infants with PDA. Photoplethysmography constitutes the optical signal in pulse oximetry. STUDY DESIGN: Stroke volume was determined echocardiographically. Pulse transit time, right hand to foot arrival time difference, and relative amplitude were measured from pulse oximeter and ECG waveforms. Photoplethysmographic parameters before and after PDA closure were compared with stroke volume. RESULTS: After PDA closure, pulse transit time to the hand and to the foot were prolonged (54.7 ± 6.7 vs 65.5 ± 9.8 ms, p < 0.001, 82.5 ± 12.8 vs 88.6 ± 10.6 ms, p = 0.03), arrival time difference decreased (27.7 ± 7.6 vs 23.1 ± 5.6 ms, p = 0.021), and relative amplitude decreased (from 2.1 ± 0.7% to 1.5 ± 0.5%, p = 0.003). The time-based photoplethysmographic parameters correlated with stroke volume. CONCLUSIONS: Photoplethysmographic waveform parameters are significantly different before and after PDA closure and the time-based parameters correlate well with stroke volume. Monitoring pulse transit time may assist in evaluation for spontaneous PDA closure or response to therapy.

Radiofrequency catheter ablation for dyssynchrony-induced dilated cardiomyopathy in an infant.

The relationship between accessory pathway-mediated ventricular preexcitation and left ventricular dyssynchrony-induced dysfunction has been described in patients with Wolff-Parkinson-White (WPW) syndrome in the absence of sustained supraventricular tachycardia (SVT). Supraventricular tachycardia in infants is usually successfully suppressed with antiarrhythmic medications, but catheter ablation has ultimately been required as definitive treatment in medically resistant cases. Catheter ablation has not been described in young infants for dyssynchrony-related dilated cardiomyopathy in the absence of SVT. We describe a case of an infant with WPW who did not have sustained supraventricular tachycardia, but who developed rapid progression of ventricular dysfunction after birth. Preexcitation could not be medically suppressed but was successfully ablated. This was followed by complete resolution of ventricular dysfunction within 2 months.

Restrictive versus nonrestrictive physiology following repair of tetralogy of Fallot: is there a difference?

BACKGROUND: Long-term outcome in repaired tetralogy of Fallot (TOF) is related to chronic pulmonary insufficiency (PI), right ventricular (RV) dilation, and deterioration of RV function. The aim of this study was to characterize clinical differences between restrictive and nonrestrictive RV physiology in young patients with repaired TOF. METHODS: Patients were prospectively enrolled from February 2008 to August 2009. Each had a clinic visit, brain natriuretic peptide assessment, exercise test, cardiac magnetic resonance study, and echocardiographic examination with assessment of regional myocardial mechanics. Consistent antegrade diastolic pulmonary arterial flow with atrial contraction identified restrictive RV physiology. RESULTS: Twenty-nine patients (median age, 12 years; range, 8-33 years; nine male patients) were studied. Twelve had restrictive RV physiology. The median time since initial TOF repair was 12 years (range, 5-27 years). Restrictive physiology appeared more prevalent after transannular patch repair and was not influenced by other demographic features. The restrictive group had more PI (46% vs 28%, P = .002), larger RV end-diastolic volumes (128 vs 98 mL/m(2), P = .046), but similar ejection fractions, brain natriuretic peptide levels, New York Heart Association classes, and exercise capacity. RV basal and mid free wall peak diastolic strain rate differed between groups, negatively correlating with exercise time and positively correlating with PI in patients with restrictive physiology. CONCLUSIONS: Restrictive RV physiology correlates with a larger right ventricle and increased PI after TOF repair but does not negatively affect other markers of myocardial health. Diastolic regional RV myocardial mechanics, particularly diastolic velocity and peak diastolic strain rate, differ for postoperative TOF patients with restrictive and nonrestrictive RV physiology; longitudinal study is necessary to understand the relationship of regional myocardial mechanics and patients' clinical status.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Fetal right-ventricular hypertrophy with pericardial effusion and maternal untreated hyperthyroidism.

Uncontrolled/untreated maternal hyperthyroidism has been associated with fetal tachycardia. We report a case of right-ventricular (RV) hypertrophy with pericardial effusion related to untreated maternal Graves' disease. A 33-year-old G4P1021 woman with uncontrolled Graves' disease presented at 29 weeks gestation with abdominal pain and vaginal bleeding. Fetal echocardiogram showed severe RV hypertrophy and a pericardial effusion. The infant was born prematurely, and initial transthoracic echocardiogram showed severe RV hypertrophy and a small pericardial effusion. The infant had clinical findings consistent with congenital thyrotoxicosis and was treated for this. Follow-up imaging at 4 weeks showed improvement of the cardiac hypertrophy and pericardial effusion. This article describes the presentation of fetal RV hypertrophy with congenital thyrotoxicosis and underscores the importance of screening for this prenatally in mothers with uncontrolled or untreated hyperthyroidism.

Children and adolescents with repaired tetralogy of fallot report quality of life similar to healthy peers.

OBJECTIVE: The study aims to evaluate and compare self-reported and parent proxy-reported quality of life (QOL) in pediatric patients with repaired tetralogy of Fallot (TOF) and determine relationships with residual disease. DESIGN: QOL was prospectively evaluated in children/adolescents with repaired TOF and parents' proxy report using the Pediatric Quality of Life Inventory Generic Core and Cardiac Module scales. The scores were compared with published self and parent proxy-reported normative data for children considered healthy, chronically ill, and with congenital heart disease. Recent clinical data were reviewed for correlations between QOL and residual disease severity. RESULTS: Twenty child-parent pairs were assessed at median age of 10.9 years (range 8.4-18.7 years). Self-report was higher than parent proxy report. Compared with peers, self-reported QOL was higher than for chronically ill children (overall QOL 85 vs. 77, P= 0.007) and similar to healthy children (85 vs. 83, P= 0.44), while proxy report by parent was similar to parents of chronically ill children (overall QOL 77 vs. 74, P= 0.035). Despite moderate pulmonary regurgitation (mean 35%) and right ventricular dilation (mean 114 mL/m(2) ), 76% had a New York Heart Association class of 1, normal B-natriuretic peptide (24 pg/mL), reasonable exercise tolerance VO(2) max% predicted (mean 77%), and preserved right ventricular ejection fraction (mean 58%, range 44-80%). Overall QOL positively correlated with child's VO(2) max% predicted, when reported by child (r = 0.47, P < 0.05) and parent proxy (r = 0.63, P < 0.05). CONCLUSIONS: QOL in children/adolescents with repaired TOF is not proportional to the severity of their residual disease. Self-reported QOL appears similar to healthy peers while parent proxy reported lower QOL. For both children and parents, QOL positively correlated with the child's exercise capacity. Therefore, comprehensive follow-up should include cardiac rehabilitation and psychosocial evaluation to ensure an active lifestyle, improve health perception, and prevent later acquired heart disease.