Remi Kowalski

Twenty-four-hour ambulatory blood pressure monitoring detects a high prevalence of hypertension late after coarctation repair in patients with hypoplastic arches.

OBJECTIVES To determine by 24-hour blood pressure monitoring the risk of hypertension late after coarctation repair in patients with arch hypoplasia. METHODS Sixty-two of 116 consecutive patients (age, ≥10 years) who had coarctation repair and were quoted subjectively by the surgeon or the cardiologist to have arch hypoplasia at the time of the repair underwent a transthoracic echocardiogram and 24-hour blood pressure monitoring. Median age at repair was 11 days (range, 6-48 days). Mean preoperative z score of the proximal transverse arch was -2.43 ± 0.46. Eight patients had a repair via sternotomy (6 end-to-side anastomoses, 2 patch repairs) and 54 had a conventional repair via thoracotomy. RESULTS After a follow-up of 18 ± 5 years, 27% of the patients (17/62) had resting hypertension and 60% (37/62) had abnormal ambulatory blood pressure. Sensitivity of high resting blood pressure in detecting an abnormal 24-hour ambulatory blood pressure was 41%. Twenty patients had arch obstruction at last follow-up. Eighteen of them (90%) had abnormal ambulatory blood pressure. None of the patients operated on with end-to-side repair via sternotomy had reobstruction compared with 33% (18/54) of those repaired via thoracotomy. CONCLUSIONS Patients with a hypoplastic arch operated via thoracotomy have an alarming prevalence of hypertension. Regular follow-up with 24-hour ambulatory blood pressure monitoring is warranted, especially in patients who have had a smaller aortic arch at the time of the initial operation.

Ross Procedure in Children: 17‐Year Experience at a Single Institution

Background The Ross procedure in children carries substantial mortality and reoperation rate. Aortic root dilatation is of concern. To prevent dilatation of the neoaortic root, but permit normal growth, we began to apply an absorbable poly‐(p‐dioxanone)‐filaments (PDS) band at the sino‐tubular (ST)‐junction. Methods and Results All children (n=100) who underwent Ross procedure during 1995–2012 were studied. Mean age at operation was 8.6±6.1 years (median 8.3 years, range 3 days to 18 years); 19 patients were younger than 1 year of age. The root replacement (n=91, Ross‐Konno procedure in 29 patients), root inclusion (n=6), and subcoronary implantation (n=3) techniques were used. Operative mortality was 6% (6/100, 4 neonates, 2 infants). Age of <1‐year at time of operation was a risk factor for early death (P<0.001). Mean follow‐up time was 7.0±4.8 years (median 7.4 years, range 5 days to 16 years). Late mortality was 4.3% (4/94). Freedom from moderate or greater neoaortic valve insufficiency (AI) at 5 and 10 years was 89% and 83%, respectively. Freedom from neoaortic valve reoperation at 5 and 10 years was 96% and 86%, respectively. Aortic dilatation to Z‐score >4 was greatest at the ST‐junction (23%, 11/48) compared to the aortic annulus (17%, 11/66) and sinuses (14%, 7/50). Since 2001, a PDS band was placed around the ST‐junction in 19 patients. Survivors with the PDS band had less AI (0 versus 20%, P=0.043) compared to survivors (n=35) without the PDS at 4.1±3 years. Conclusions The Ross procedure in children can be performed with acceptable results. Children younger than 1 year of age have higher mortality, but not an increased autograft reoperation rate. Stabilization of the ST‐junction may reduce AI.

Moderately hypoplastic arches: do they reliably grow into adulthood after conventional coarctation repair?

UNLABELLED To determine whether conventional coarctation repair results in sustained growth of hypoplastic transverse arches, we examined the follow-up of 20 patients operated through a thoracotomy between 1990 and 1995 who had available serial echocardiographic examinations. Mean age at operation was 8.6+/-5.7 days. In the distal transverse arch, maximum change was observed in the early postoperative period and sustained growth was observed thereafter. At last follow-up, no patients had Z-scores of less than -2. In contrast, only minimal growth occurred in the proximal transverse arch (mean Z-score diameter before and after repair: -1.87+/-0.12 vs. -1.66+/-0.09; P=0.05) in the postoperative period. At last follow-up, seven patients (35%) kept a diameter Z-score of less than -2, and 5 of them had a gradient of 15 mmHg (P=0.01). No correlation was found between the size of the proximal arch at last follow-up and its size before repair or technique used. CONCLUSION Patients with moderately hypoplastic arch treated by conventional coarctation repair have adequate growth of the distal arch demonstrated at long-term follow-up, but one-third of them keep a small proximal arch. This subset of patients is at risk of developing hypertension and may warrant further investigation.

Kawasaki disease and cardiovascular risk: a comprehensive review of subclinical vascular changes in the longer term.

Studies of subclinical vascular changes post‐Kawasaki disease indicate that, in general, individuals with a history of coronary artery aneurysms have increased carotid intima–media thickness, evidence of endothelial dysfunction, and increased arterial stiffness, possibly indicative of heightened cardiovascular risk. The results are less consistent for low‐risk groups.

Risk factors and clinical features of acute pulmonary embolism in children from the community.

BACKGROUND AND AIMS Current clinical decision rules for pulmonary embolism are based on adult populations and have not been validated in children. The objective was to identify and evaluate clinical features for a first lifetime episode of pulmonary embolism in children presenting to the emergency department. MATERIALS AND METHODS We present a case-control study of children (≤18years) presenting to the emergency department of the Royal Childrens Hospital, Melbourne between November 2007 and February 2015. Children with radiologically proven pulmonary embolism formed the case group, whilst children in whom there was a clinical suspicion of pulmonary embolism but negative diagnostic imaging formed the control group. Charts, electronic medical and imaging records of both cases and controls were reviewed and analysed. RESULTS There were a total of 50 patients in this study (11 cases and 39 controls). Current or recent (within three months) use of the oral contraceptive pill was the most significant risk factor in our study (odds ratio 14.667, 95% confidence interval 3.001-71.678, P<0.001). Most other features failed to discriminate between cases and controls, although there was a trend towards increased heart rate in cases. CONCLUSIONS Pulmonary embolism is perhaps the most common presenting spontaneous venous thromboembolism in the community and teenage girls on the oral contraceptive pill are most at-risk amongst children. The clinical signs and symptoms are often non-specific. Additional larger studies are required to determine the significance and magnitude of potential clinical predictors identified in this study. This may lead to derivation of a paediatric-specific pre-test probability tool.

Beyond the aorta: partial transmission of reflected waves from aortic coarctation into supra-aortic branches modulates cerebral hemodynamics and left ventricular load

Wave reflection from the site of aortic coarctation produces a reflected backward compression wave (BCW) that raises left ventricular (LV) afterload. However, not all reflected wave power will propagate back to the LV. This study investigated the hypothesis that the BCW is partially transmitted into supra-aortic vessels as a forward wave and explored the consequences of this phenomenon for cerebral and LV haemodynamic load. In eight sheep, high fidelity pressure and flow were measured in the aortic trunk (AoT) and brachiocephalic trunk (BCT, the single supra-aortic vessel present in sheep) at baseline and during two levels of proximal descending aortic constriction. Wave power analysis showed that aortic constriction produced not only a BCW in the AoT, but also a second forward compression wave (

Robust and practical non-invasive estimation of local arterial wave speed and mean blood velocity waveforms

\mathrm{FCW}_{2})

High Prevalence of Hypertension and End-Organ Damage Late After Coarctation Repair in Normal Arches

FCW2) in the BCT that augmented pressure and flow after the initial forward compression wave (