Evolution in the management of aorta to left ventricular tunnel in a national congenital cardiology centre.

An aorto-ventricular tunnel is a rare congenital cardiac defect, where a channel connects the lumen of the ascending aorta to the left or right ventricle. Four patients presented with an aorto-left ventricular tunnel over two decades at a median age of 8 months (range 0.1-10 months). Two patients (50%) had associated cardiac anomalies including hypoplastic left heart syndrome and left ventricular noncompaction/hypertrophic cardiomyopathy with aortic/pulmonary valve dysplasia in one patient each. Although traditionally surgical treatment has addressed this problem, management has evolved to transcatheter closure with excellent outcomes in appropriately selected patients at our national centre.

The impact of compliance on Stage 2 uni-ventricular heart circulation: An experimental assessment of the Bidirectional Glenn.

The Bidirectional Glenn (BDG) or cavopulmonary connection is typically undertaken to volume unload the single ventricle in an effort to preserve ventricular and atrioventricular valve function. The geometry of this surgical palliation has been shown to influence the fluid energy loss as well as the distribution of flow that enters through the superior vena cava. In-vitro and in-silico studies to date have been performed on rigid wall models, while this investigation looks at the impact of flexible thin walled models versus rigid walls. Rigid and compliant models of two patient-specific Glenn geometries were fabricated and tested under various flow conditions, within a biosimulator capable of replicating patient specific flow conditions. It was found that the compliant models exhibit greater levels of energy loss compared to the rigid models. Along with these findings greater levels of turbulence was found in both compliant models compared to their rigid counterparts under ultrasound examinations. This shows that vessel compliance has a significant impact on the hemodynamics within hypoplastic left heart syndrome.

Cyanotic Congenital Heart Disease Modes of Presentation and Prenatal Detection

Prenatal detection of structural congenital heart disease (CHD) optimises cardiovascular stability pre-operatively and post-operative outcomes. We compared prenatal detection rates of critical CHD in units offering universal fetal anomaly scans with those offering imaging to selected women. One hundred and thirteen infants met inclusion criteria. The overall pre-natal detection rate for critical CHD was 57% of liveborn infants. It was 71% (57/80) in hospitals who offered a universal anomaly scan and 29% (9/31) in centres offering a limited service. Postnatal diagnosis was associated with PICU admission (p=0.016) and preoperative mechanical ventilation (p=0.001). One-year mortality was 10 fold higher in the postnatally diagnosed group 15% vs 1.55% (p=0.0066). There is a significant disparity between centres offering universal anomaly versus selective screening. Prenatal detection confers advantage in terms of pre-operative stability and one year survival. Failure to deliver an equitable service exposes infants with CHD to avoidable risk.

Kawasaki Disease - A Review of Treatment and Outcomes in an Irish Paediatric Cohort 2010-14.

Diagnosis of Kawasaki Disease (KD) can be challenging due to lack of a diagnostic test, and some children present with 'incomplete' KD when not all diagnostic criteria are met. Treatment with intravenous immunoglobulin (IVIG) and aspirin reduces the risk of coronary artery complications. There is sub-group of patients who are resistant to IVIG/aspirin therapy and are at increased risk of complications. Recent evidence suggests that additional treatment of this high-risk group with corticosteroids is beneficial in reducing this risk. We examine the treatment and coronary artery outcomes, by retrospective review of medical records, of a cohort of 32 paediatric patients with KD admitted to a single Irish tertiary centre from January 2010-December 2014. Twenty-eight percent of patients (9/32) had an incomplete diagnosis of KD; these patients received IVIG later compared to those with a complete KD diagnosis. 15/32 (47%) had abnormal echocardiogram findings in the acute phase, 8/32 (25%) had echocardiogram abnormalities at 6-week follow-up, and 4/32 (12.5%) had persisting abnormalities. This study highlights the potential for adverse outcome in KD, the difficulty in diagnosis in 'incomplete' cases, and the need to identify children at higher risk for adverse outcome where adjunctive therapies would be most beneficial.

3D printing of severe hypertrophic cardiomyopathy in a child with Rasopathy.

We describe the use of 3D printing in conjunction with echocardiography in assessing hypertrophic cardiomyopathy in a boy with Rasopathy. 3D printing may supplement conventional imaging including echocardiography and MRI in the evaluation of hypertrophic cardiomyopathy.

Dilated cardiomyopathy secondary to vitamin D deficiency and hypocalcaemia in the Irish paediatric population: A case report.

We identified three infants with dilated cardiomyopathy (DCM) secondary to severe vitamin D deficieny and hypocalcaemia. All infants were exclusively breast fed, from dark skinned ethnic backgrounds, born and living in Ireland. None of these pregnant mothers or infants received the recommended vitamin D supplementation. Each infant presented in heart failure and required inotropic support as well as calcium and vitamin D replacement. Cardiac function subsequently improved. This highlights the public health issue that many high risk pregnant mothers and infants are not receiving the recommended vitamin D supplementation.

Accuracy of 64-section MDCT in the diagnosis of cruciate ligament tears.

AIM: To evaluate the accuracy of non-arthrographic 64-section multidetector computed tomography (CT) in the assessment of cruciate ligament tears. A secondary goal was to determine its accuracy in the diagnosis of additional soft-tissue injuries around the knee. MATERIALS AND METHODS: Forty consecutive outpatients underwent same-day magnetic resonance imaging (MRI) and 64-slice multidetector CT (MDCT) of the knee in this prospective study. MDCT images were independently evaluated for integrity of the anterior (ACL) and posterior cruciate ligaments (PCL), medial and lateral menisci, and medial and lateral collateral ligaments. Recognised secondary signs of ACL tears were also documented. MRI images were subsequently assessed by two radiologists and a consensus reached. RESULTS: The sensitivity of MDCT for ACL tears was 87.5-100%, with a specificity of 100%. The presence of one or more secondary signs of ACL tears on MDCT had a sensitivity of 50-87.5% with a specificity of 100%. The sensitivity of MDCT for PCL tears was 0-25% with a specificity of 100%. The sensitivity for meniscal tears was 9.1-23.1% with a specificity of 96.3-100%. CONCLUSION: 64-section MDCT has very high sensitivity and specificity for ACL tears and, as on MRI, secondary signs, such as buckling of the PCL, are also useful in their diagnosis. MDCT has a low sensitivity for other soft-tissue injuries at the knee; however, its high specificity indicates that apparent PCL, meniscal, and collateral ligament tears can reliably be treated as true-positive findings.

Is diffusion-weighted MRI sufficient for follow-up of neuroendocrine tumour liver metastases?

AIM: To assess if diffusion-weighted imaging (DWI) alone could be used for follow-up of neuroendocrine hepatic metastases. MATERIAL AND METHODS: This was a retrospective study, approved by the institutional review board. Twenty-two patients with neuroendocrine liver metastases who had undergone more than one liver magnetic resonance imaging (MRI) examination, (including DWI and using hepatocyte-specific contrast medium) were evaluated. Up to five metastases were measured at baseline and at each subsequent examination. The reference standard measurement was performed on the hepatocyte phase by one reader. Three independent readers separately measured the same lesions on DWI sequences alone, blinded to other sequences, and recorded the presence of any new lesions. RESULTS: The longest diameters of 317 liver metastases (91 on 22 baseline examinations and a further 226 measurements on follow-up) were measured on the reference standard by one reader and on three b-values by three other readers. The mean difference between DWI measurements and the reference standard measurement was between 0.01-0.08 cm over the nine reader/b-value combinations. Based on the width of the Bland and Altman interval containing approximately 95% of the differences between the reader observation and the mean of reference standard and DWI measurement, the narrowest interval over the nine reader/b-value combinations was -0.6 to +0.7 cm and the widest was -0.9 to 1 cm. In the evaluation of overall response using Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 criteria, the weighted kappa statistic was between 0.49 and 0.86, indicating moderate-to-good agreement between the reference standard and DWI. CONCLUSION: The visualisation and measurement of hepatic metastases using DWI alone are within acceptable limits for clinical use, allowing the use of this rapid technique to restage hepatic disease in patients with neuroendocrine metastases.

Predictive value of PI-RADS classification in MRI-directed transrectal ultrasound guided prostate biopsy.

AIM: To correlate the results of transrectal ultrasound (TRUS)-guided targeted prostate biopsies (performed in the setting of at least one previous negative biopsy) with the Prostate Imaging Reporting and Data System (PI-RADS). MATERIAL AND METHODS: Fifty-two patients (mean age 64 years, range 52-76 years), with previous negative prostate biopsy underwent magnetic resonance imaging (MRI)-directed TRUS-guided targeted and sectoral biopsy. A retrospective review of MRI examinations was carried out, blinded to biopsy results. PI-RADS scores (T2, diffusion-weighted imaging [DWI] and overall) were assigned on a per lesion basis, and localised to sextants. The scores were correlated with biopsy results, and the positive predictive values (PPV) of PIRADS scores for positive biopsies were calculated. RESULTS: Overall, biopsies were positive in 23/52 (44.2%) patients. Eighty-one areas were targeted in 52 patients. On a per lesion basis, there was significant correlation between positive targeted biopsy and both T2 and overall PI-RADS score (p<0.001). The correlation between biopsy and DWI score was significant for peripheral zone tumours only, not for transitional zone tumours. The PPV of overall PI-RADS scores of 3, 4, and 5 were 10.6%, 44%, and 100%, respectively. The PPV of T2 PI-RADS scores of 3, 4, and 5 were 19.6%, 60%, and 100%, respectively. The PPV of DWI PI-RADS scores of 3, 4, and 5 were 50%, 27.3%, and 33%, respectively. When transitional tumours were excluded, the PPV of DWI PI-RADS 3, 4, and 5 were 40%, 43%, and 78%. CONCLUSION: The PIRADS score provides an effective framework for determining the likelihood of prostate cancer on MRI. The DWI PI-RADS score correlates well with the presence of peripheral zone tumour on targeted biopsy, but not with transitional zone tumours.

First reported use of drug-eluting bioabsorbable vascular scaffold in congenital heart disease.

The aim of catheter intervention for vascular stenosis is the restoration of lumen area and optimization of distal blood flow. In pediatric practice, this has traditionally been a compromise between less effective balloon angioplasty and bare metal stent insertion with its attendant limitations of size. Bioabsorbable stents offer short-term relief of stenosis, radial support of the healing lesion, return of endothelial function and crucially, in children, the potential for long-term growth. Initial experience, in pediatric practice, with metal-based bioabsorbable stents was relatively disappointing with frequent restenosis secondary to early reabsorption. Design modifications resulting in polymer-based, drug eluting, bioabsorbable vascular scaffolds (BVS) have reportedly overcome some of these faults. We describe the first reported use of a drug eluting BVS in three patients with: (1) A newborn with severe right pulmonary artery (RPA) stensosis post repair of type two common arterial trunk. (2) A child with pulmonary atresia/ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), and (3) An infant with severe left pulmonary artery (LPA) stenosis in the setting of an LPA sling.

Myocardial ischaemia following cocaine and adrenaline exposure in a child during an ophthalmological procedure.

We report a 23-month old girl who presented with bilateral epiphora who underwent bilateral lacrimal probing and syringing, during which a cocaine adrenaline solution was used. Two hours after the procedure she developed acute pulmonary oedema secondary to myocardial ischaemia. The patient was treated with intravenous glyceryltrinitrate and milrinone infusions; cardiac enzymes and left ventricular function normalised over the subsequent 72 hours. Topical administration of cocaine and adrenaline solution may have dangerous systemic cardiac effects and should always be used judiciously.

Should abdominal sequences be included in prostate cancer MR staging studies?

OBJECTIVES: Prostate cancer staging MR examinations commonly include abdominal sequences to assess for non-regional (common iliac or para-aortic) nodal metastasis. In our experience the diagnostic yield of this is limited, but incidental findings are frequent, often necessitating further investigations. The aim of this study is to assess the diagnostic utility of abdominal sequences in routine prostate cancer MR staging studies. METHODS: Findings on abdominal sequences of consecutive MRI prostate studies performed for staging newly diagnosed prostate cancer between September 2011 and September 2013 were reviewed with respect to adenopathy and additional incidental findings. Results were correlated with Gleason grade and serum prostate-specific antigen (PSA) level in each case. RESULTS: 355 MRI prostate examinations were reviewed. 4 (1.1%) showed enlarged non-regional lymph nodes. Incidental findings were found in 82(23.1%) cases, neccessitating further investigation in 45 (12.7%) cases. Enlarged non-regional nodes were associated with higher PSA level and Gleason grade (p=0.007, p=0.005 respectively). With a combined threshold of PSA > 20 ng/mL and/or Gleason grade ≥ 8 the sensitivity, specificity, PPV and NPV were 100, 60, 3 and 100% respectively for predicting the presence of non-regional adenopathy. CONCLUSIONS: Routine abdominal sequences are of very low yield in routine prostate cancer MR staging, frequently resulting in incidental findings requiring further work-up and should be reserved for high-risk cases. Our experience supports the use of an abdominal staging sequence in high-risk cases only.

Mode of delivery in pregnancies complicated by major fetal congenital heart disease: a retrospective cohort study.

OBJECTIVE: To determine the mode of delivery in pregnancies complicated by complex fetal congenital heart disease (CHD). STUDY DESIGN: Five-year retrospective cohort study at a tertiary fetal medicine center (2007 to 2011). Cases of complex fetal CHD (n=126) were compared with 45 069 non-anomalous singleton infants ⩾500 g to determine rates of emergency intrapartum cesarean section (CS), preterm delivery and induction of labor. RESULT: Intrapartum CS is significantly higher in fetal CHD than non-anomalous controls (21% vs 13.5%, odds ratio (OR) 1.7, 95% confidence interval (CI): 1.0 to 2.7; P=0.035), predominantly related to CS for non-reassuring fetal status (OR 2.2, 95% CI: 1.1 to 4.1; P=0.022). Although fetal CHD did not increase emergency CS rates in nulliparous women, CS was significantly increased in multiparous pregnancies (OR 2.4, 95% CI: 1.8 to 4.6; P=0.014). Rates of preterm delivery (OR 3.4, 95% CI: 2.0 to 5.4; P<0.0001) and induction of labor (OR 1.9, 95% CI: 1.3 to 2.9; P=0.001) were higher in the CHD cases. CONCLUSION: Emergency CS is increased in fetal CHD, attributed to a higher rate of CS for non-reassuring fetal status and seen mostly in multiparous women.

Hemoptysis from bronchial varices associated with pulmonary vein stenosis: role of surgical repair.

We present the case of a 6-year-old child who presented with an episode of life threatening hemoptysis. Investigations revealed multiple areas of endobronchial varices and abnormal pleural vessels as well as severe left pulmonary vein stenosis and an atrial septal defect (ASD). After extensive work up and consultation he underwent repair of the left pulmonary vein using a sutureless technique and ASD closure. This resulted in a marked improvement in the appearances of the left lung. The bronchial varices in the right lung remain unchanged. No further hemoptysis has occurred and the child continues to be monitored.

Berry syndrome in association with familial limb malformation.

We describe a newborn boy diagnosed with Berry syndrome consisting of a distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, and interruption of the aorta. The child was noted to have reduplication of the right thumb. The child's mother had a claw malformation of her left hand but a normal cardiovascular status. Genetic analysis for TBX5 and SALL4 mutations were negative in both the patient and his mother. This case describes the first ever report of Berry syndrome in an infant with reduplication of the right thumb and familial limb malformation.

Parvovirus B19 infection associated with dilated cardiomyopathy in patients with previous anthracycline exposure.

We describe two children with previous anthracycline exposure for cancer who presented with acute decompensated left ventricular dysfunction. Both patients had evidence of dilated cardiomyopathy and required mechanical ventilation and inotropic support. Parvovirus B19 was detected by polymerase chain reaction of the blood. After several weeks of ventilation and inotropic support, both patients were weaned from ventilation and managed with oral carvedilol, ACE inhibition, and diuretics. Acute left ventricular decompensation in patients following anthracycline exposure may not be solely attributed to drug exposure, and viral etiologies should be considered.

Long-term support with milrinone prior to cardiac transplantation in a neonate with left ventricular noncompaction cardiomyopathy.

A 2-week-old female infant presented with acute decompensated left ventricular failure. Echocardiography diagnosed left ventricular noncompaction cardiomyopathy with dilated phenotype and a left ventricular shortening fraction <10%. The infant was mechanically ventilated for 2 weeks and then successfully extubated. She was maintained on intravenous milrinone for 6 months until she underwent successful orthotopic heart transplantation. Young children can be supported with inotropes for prolonged periods while awaiting heart transplantation.

The reproducibility of transcranial Doppler middle cerebral artery velocity measurements: implications for clinical practice.

Use of transcranial Doppler (TCD) to diagnose vasospasm has been criticised. We examined reproducibility of TCD middle cerebral artery (MCA) velocity measurements. Thirty-six healthy adult volunteers were recruited. Four operators, two experienced and two inexperienced, participated. MCA velocity was measured twice by one operator and once by a second operator. Mean (95% limits of agreement) interoperator agreement was 2.4(+/-36.7) cm/s. Experienced vs. inexperienced, inexperienced vs. inexperienced, and experienced vs. experienced operators were -2.8(+/-39.3), -5.6(+/-40.1), 1.8(+/-22.1) cm/s, respectively. Intraoperator agreement across all operators, experienced and inexperienced were -0.5(+/-16.9), -1.6(+/-19.3), 0.7(+/-13.7) cm/s, respectively. Interoperator limits of agreement for experienced operators were almost half that of inexperienced operators. Intraoperator reproducibility was much better, regardless of level of experience, but aberrant results did occur even with experienced operators. If TCD measurements are used to guide management it is essential that operators are adequately trained, and readings repeated before potentially harmful treatments are instituted.