Distal Transverse Arch to Left Carotid Artery Ratio Helps to Identify Infants With Aortic Arch Hypoplasia.

BACKGROUND: Aortic coarctation (CoA) with concomitant aortic arch hypoplasia (AAH) is associated with an increased risk of hypertension after surgical repair. The differentiation of CoA with or without AAH may be critical to delineate the ideal surgical approach that best ameliorates postoperative hypertension. Since 2000, we have defined CoA with AAH when the diameter of the distal transverse aortic arch is equal to or less than the diameter of the left carotid artery. We hypothesized that, based on our definition, aortic tissue from infants having CoA with AAH would demonstrate distinct genetic expression patterns as compared with infants having CoA alone. METHODS: From 6 infants (AAH, 3; CoA, 3), an Affymetrix 1.0 genome array identified genes in the coarctation/arch region that were differentially expressed between infants having CoA with AAH versus CoA alone. Reverse transcription polymerase chain reaction validated genetic differences from a cohort of 21 infants (CoA with AAH, 10; CoA, 11). To evaluate the clinical outcomes based on our definition of CoA with AAH, we reviewed infants repaired using this algorithm from 2000 to 2010. RESULTS: Microarray data demonstrated genes differentially expressed between groups. Reverse transcription polymerase chain reaction confirmed that CoA with AAH was associated with an increased expression of genes involved in cardiac and vascular development and growth, including hepsin, fibroblast growth factor-18, and T-box 2. The clinical outcomes of 79 infants (AAH, 26; CoA, 53) demonstrated that 90.1% were free of hypertension at 13 years when managed with this surgical strategy. CONCLUSIONS: These findings provide evidence that the ratio of the diameter of the distal transverse arch to the left carotid artery may be helpful to identify CoA with AAH and, when used to delineate the surgical approach, may minimize hypertension.

Successful Treatment of a Neonate with Idiopathic Persistent Pulmonary Hypertension with Inhaled Nitric Oxide via Nasal Cannula without Mechanical Ventilation.

We report a case study of a term neonate presenting with oxygen desaturation without respiratory distress or acidosis, despite receiving 100% oxygen through a nasal cannula. Echocardiogram showed evidence of persistent pulmonary hypertension of the newborn (PPHN). She was successfully treated with inhaled nitric oxide (iNO) via nasal cannula without requiring mechanical ventilation. In a term neonate with idiopathic PPHN with adequate respiratory drive without any parenchymal lung disease, noninvasive methods of iNO delivery may treat the condition without the complications associated with mechanical ventilation.

Surgical strategy for aortic coarctation repair resulting in physiologic arm and leg blood pressures.

OBJECTIVE: The goal of aortic coarctation repair is laminar aortic blood flow resulting in a negative or absent arm:leg blood pressure (BP) gradient. Despite satisfactory relief of coarctation, associated arch hypoplasia can result in residual obstruction and postoperative upper body hypertension. INTERVENTION: We devised a surgical strategy to create a tension-free anastomosis with a diameter as large as both the adjacent proximal and distal aorta using a radically extended end-to-end anastomosis via sternotomy and/or thoracotomy. Sternotomy is chosen when there is significant transverse arch hypoplasia defined as a distal transverse arch ≤ diameter of the left carotid artery, presence of a common brachiocephalic trunk, or coexisting intracardiac lesion requiring repair. Thoracotomy is used in all other cases. RESULTS: From 2000 to 2008, 95 consecutive patients were repaired using this approach, 35 with sternotomy and 60 with thoracotomy. At a mean follow-up of 50 ± 23 months, mean systolic BP was 94 ± 10 mm Hg, and 84% of patients had no residual arm:leg BP gradient. Mean arm:leg BP gradient was not statistically different between groups (-8.5 ± 15 sternotomy and -7.0 ± 10 mm Hg thoracotomy, P= .7). With Doppler echocardiography, 96% of patients demonstrated normal early diastolic reversal of blood flow in the descending thoracic aorta. CONCLUSIONS: For aortic coarctation repair in infancy, a strategy designed to directly address aortic arch hypoplasia results in excellent intermediate-term results with normal BP, physiologic arm:leg BP relationship, and near normal descending aortic blood flow velocities by Doppler.

Coronary artery calcifications in the long-term follow-up of Kawasaki disease.

BACKGROUND: To determine if detection of coronary artery calcifications in patients with Kawasaki disease may serve as a noninvasive predictor of future coronary artery events. METHODS: A prospective, cohort pilot study that included 18 patients with Kawasaki disease > 1 year from the acute disease was performed including 9 patients with coronary abnormalities during the acute illness (Group 1) and 9 without coronary abnormalities (Group 2). Patients were classified by echocardiography as having none, resolved, or residual coronary artery abnormalities. Electron beam computed tomography (EBCT) scans were completed using the Agatson coronary calcium scoring system. Intermediate follow-up was performed 2.5 years after EBCT to determine if clinically significant coronary artery events (myocardial infarction or sudden death) had occurred. RESULTS: Late echocardiographic abnormalities corresponded with the early echocardiographic abnormalities in 5 of 9 patients (P = .029) in Group 1. The late echocardiographic abnormalities significantly correlated with detection of calcifications by EBCT in 4 of 5 patients (95% CI 28%-99%). One patient with residual coronary abnormalities and coronary artery calcifications with the highest calcium score subsequently had a sudden death. Detection of coronary artery calcifications may be predictive of sudden death (P = .056). No residual echocardiographic abnormalities, coronary artery calcifications, or coronary artery events occurred in Group 2 patients. CONCLUSIONS: Patients with Kawasaki disease with residual coronary abnormalities show EBCT evidence of coronary artery calcifications. Detection of coronary artery calcifications may be useful for risk stratification in the long-term management of patients with Kawasaki disease.