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High blood pressure (BP) in childhood is a recognised precursor of elevated cardiovascular risk in adulthood. Brachial BP is normally used for clinical decision making, but central BP may be a better marker of pressure load on the heart. There is a paucity of validated non-invasive, automated devices for estimating central BP in children and adolescents. In this study, we compared the WatchBP Office Central (a Type 2 central pressure estimation device) against a high-fidelity micromanometer in the ascending aorta of anaesthetised patients undergoing clinically-indicated catheterisation (n = 15, age 4-16 years). As a secondary aim, central systolic BP (cSBP) was also compared to two non-invasive estimation methods in 34 awake patients undergoing routine cardiac MRI (age 10-18 years). WatchBP substantially overestimated cSBP compared to the intra-arterial gold-standard reference (26.1 ± 7.4 mmHg), and recruitment was terminated at n = 11 (included in the analysis) due to high statistical certainty that the device would not pass the validation criteria of 5±8 mmHg. WatchBP cSBP was also substantially higher than values obtained from a phase contrast MRI method (11.8 ± 7.9 mmHg) and the SphygmoCor XCEL (13.5 ± 8.9 mmHg) in the awake patient group, which translate to 21-23 mmHg on average after accounting for known/estimated biases in these non-invasive comparators. Compared with invasive central diastolic and systolic BPs, the brachial measures from WatchBP yielded errors of 0.1 ± 5.6 and 12.5 ± 6.0 mmHg respectively. We conclude that the WatchBP substantially overestimates cSBP in children and adolescents. These findings reinforce the need for central BP-measuring devices to be further developed and validated in this population., (© 2024. The Author(s).)

Background: Central aortic SBP (cSBP) may have superior prognostic value compared with peripheral SBP (pSBP), but noninvasive cSBP measurement techniques have not been formally validated in children and adolescents., Method: This study assessed the accuracy of two automated devices and the radial tonometry/transfer function method (RT-TF) for estimating central pressures and pulse pressure amplification (PPA) in this population, with adherence to validation guidelines for central pressure devices. In 69 children/adolescents aged 3-18 years undergoing clinically indicated aortic catheterization, high fidelity ascending aortic cSBP was measured with a micromanometer-tipped wire and compared with values from SphygmoCor XCEL, Mobil-O-Graph (systolic/diastolic calibration, MoG-C1, or mean/diastolic calibration, MoG-C2) and RT-TF. Reference intra-arterial pSBP was derived from the tonometry pulse calibrated to central mean/diastolic pressures., Results: XCEL, MoG-C1 and MoG-C2 overestimated cSBP by 7.9 ± 6.8 mmHg (mean ± SD), 5.7 ± 10.3 mmHg, and 19.1 ± 14.9 mmHg, exceeding the validation cut-off (5 ± 8 mmHg). Brachial pSBP was also overestimated by XCEL (10.9 ± 8.4 mmHg) and Mobil-O-Graph (11.5 ± 12.3 mmHg). By contrast, central and brachial diastolic pressures were underestimated by the automated devices, albeit mostly within acceptable limits; pulse pressures were, therefore, substantially overestimated. Central-brachial PPA (4.5 ± 4.4 mmHg) was overestimated by XCEL (8.7 ± 3.2 mmHg) and MoG-C1 (11.1 ± 6.4 mmHg), but underestimated by MoG-C2 (-3.0 ± 6.6 mmHg). Given accurate pulse calibration, RT-TF achieved acceptable accuracy for cSBP (-0.2 ± 4.6 mmHg) and central-radial PPA (1.9 ± 5.1 mmHg)., Conclusion: In conclusion, XCEL and Mobil-O-Graph overestimated pSBP and cSBP in children and adolescents. cSBP can be obtained via the same transfer function used in adults, but accurate pressure pulse calibration is critical.Video Abstracts: http://links.lww.com/HJH/B222.

Congenital coronary arterial fistula is a rare cause of congestive heart failure in neonates. We describe a neonate who developed early symptoms because of an unusually large left coronary artery to right ventricular fistula. Computed tomography and coronary angiography delineated anatomy and facilitated successful surgical closure., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Pulmonary artery stenosis is a challenging problem in the management of congenital heart disease. Untreated pulmonary artery stenosis may contribute to increased mortality and morbidity, and lead to suboptimal results following surgical repair of congenital heart disease. Intravascular stent implantation has emerged as one of the preferred treatment options for pulmonary artery stenosis. However, issues regarding the effectiveness and complications of stent implantation for pulmonary artery stenosis need to be identified. In addition, difficulties of stent implantation in the paediatric setting, as a consequence of small vessel size and subsequent vessel growth, are also important considerations. This review will evaluate the short and long-term effectiveness, the outcomes and complications, and discuss the potential problems of stent implantation for pulmonary artery stenosis., (Copyright © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Increasing numbers of children are surviving into adulthood following surgery to correct or palliate congenital heart disease. This surgery can occasionally result in long-standing elevated right heart pressures and chronic hepatic venous congestion leading to cardiac cirrhosis. We report the first paediatric case of hepatocellular carcinoma in the setting of cardiac cirrhosis. A 13-year-old girl developed inoperable hepatocellular carcinoma and was treated with transarterial embolization with lipiodol and doxorubicin eluting microbeads. Promoting awareness of this association, even in younger patients, will hopefully result in better surveillance and screening of hepatic complications in survivors of complex cardiac surgery., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Objectives: To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants., Background: Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants., Methods: Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent periventricular muscular VSD closure under transesophageal echo (TEE) guidance., Results: Eight infants underwent the procedure. The median age was 14 weeks (2-41) with median weight of 4 kg (3-6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4-10 mm). A single device (6-12 mm) was deployed in each patient, the size chosen being 1-2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5-66) post device implantation revealed no patients with a hemodynamically significant shunt., Conclusion: Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass., (Copyright 2008 Wiley-Liss, Inc.)

Although transesophageal echocardiography is often used for guidance during transcatheter interventions, few data exist regarding the use of the newer modality of intracardiac echocardiography. This brief report summarizes our single center experience using intracardiac echocardiographic guidance during transcatheter interventional procedures for congenital heart disease.

Objective: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition., Methods: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed., Results: The median age at operation was 28 months (range 2 months to 16.3 years). Associated defects included ventricular septal defect 40, pulmonic stenosis 13 and pulmonary atresia 16. Twenty-six patients underwent an arterial switch operation combined with a Senning procedure while 20 patients underwent combined Rastelli and Senning procedures. Before the double switch, 12 patients had required pulmonary artery banding and 21 patients had systemic to pulmonary artery shunts. The median duration of stay in the intensive care unit was 3.5 days (range 2-60 days) and the median duration of total hospital stay was 8 days (range 5-60 days). There were no hospital deaths; one patient died 5 months after discharge due to an arrhythmogenic cardiac arrest during a median follow-up of 24 months [long-term survival 98% (95% CI 89-100%)]., Conclusions: The double switch operation may be performed with excellent hospital and long-term survival. The theoretical advantages of this procedure which enables the morphologic left ventricle and mitral valve to support a systemic pressure load must be established by careful follow-up of these patients.

Patients who suffer from an embolic event often undergo placement of an inferior vena caval filter. Few data are available regarding treatment of this patient population with concurrent right-to-left shunting across an atrial communication. We report four cases of transcatheter device closure of an atrial communication across an inferior vena cava filter., (Copyright 2003 Wiley-Liss, Inc.)

Ultrasound imaging is frequently used for diagnostic purposes or guidance during procedures in the pediatric and congenital cardiac catheterization laboratory. As new imaging modalities emerged, many interventional cardiologists rather than noninvasive specialists are now performing the ultrasound imaging as part of the catheterization. The focus of this discussion will be to detail the technique and application of echocardiography by the interventional cardiologist., (Copyright 2003 Wiley-Liss, Inc.)

Patients with complex congenital heart disease may have pulmonary artery stenoses that are either congenital or associated with scarring following surgical procedures. This study evaluates cutting balloon angioplasty for small-vessel pulmonary artery stenoses resistant to standard balloon angioplasty. Between October 1998 and December 1999, patients were enrolled in an FDA-approved compassionate-use protocol. During four catheterizations, there were seven lesions found resistant to standard balloon angioplasty (mean lesion diameter was unchanged: 1.8 mm +/- 0.8 mm to 1.9 +/- 0.8 mm). A cutting balloon was inflated twice in each of these lesions. Standard balloon angioplasty was then repeated. Final mean lesion diameter was increased significantly (1.9 mm +/- 0.8 mm to 3.8 +/- 1.3 mm; P

Two patients with pulmonary atresia and intact ventricular septum each underwent early palliative surgery with a pulmonary valvotomy and an ascending aorta to pulmonary artery shunt. Adequate right ventricular growth and relief of pulmonary stenosis rendered the shunts unnecessary. The shunts were successfully occluded percutaneously with Gianturco coils.

To evaluate the potential for hyperbaric oxygen (HBO) to produce transient improvement in function in areas of myocardium ischemic at rest (hibernating myocardium), 24 patients were studied within 1 week of acute myocardial infarction. Results were compared with single-photon emission computed tomography (SPECT) thallium-201 exercise scintigraphy. Echocardiography demonstrated improved contraction following HBO in 20 of 62 damaged left ventricular segments in 12 of 24 patients. Thirteen of the 28 segments and 9 of the 14 patients showing reversible ischemia on SPECT imaging showed improvement with HBO. There were eight segments with apparently normal resting contraction that showed a reversible thallium defect. Of 42 segments with fixed contraction abnormalities following HBO, eight had reversible thallium defects, four had normal thallium kinetics, and 30 had fixed thallium defects. Thus hyperbaric oxygen can demonstrate improvement in function in some segments of left ventricle after infarction. There is some overlap with viability as determined by thallium studies, but the two techniques may be complementary in describing myocardial ischemia.

Idioventricular rate, QRS width, site of block and responses to ventricular overdrive pacing were studied in 29 patients with chronic complete heart block in an attempt to distinguish patients liable to syncopal attacks. Ten patients were asymptomatic, 5 gave a history of presyncope and 14 of syncope. Although the mean idioventricular rate of the 10 patients in the narrow QRS group (40.0 per min) was significantly faster than that of the 19 patients in the wide QRS group (35.5 per min; P less than 0.02), the rate did not distinguish symptomatic patients in either the narrow or the wide QRS groups. His bundle studies of the site of block were also unhelpful. Overdrive right ventricular pacing at increasing rates was used to assess ventricular escape times which were not significantly different in symptomatic and asymptomatic wide QRS patients. There was, however, a significant difference in the product of maximum ventricular escape time X overdrive cycle length between symptomatic and asymptomatic narrow QRS patients after 30 s overdrive (3.850+/-1.670 vs 1.070+/-0.475; P less than 0.01); 60 s overdrive (5.020+/-2.170 vs 1.240+/-0.515; P less than 0.01) and 120 s overdrive (6.040+/-2.900 vs 1.460+/-0.275; P less than 0.01) which may have clinical predictive values.

Idioventricular rate, QRS width, site of block and responses to premature ventricular stimulation were studied in 23 patients with chronic complete heart block in an attempt to distinguish patients liable to syncopal attacks. Seven patients were asymptomatic, five gave a history of presyncope and 11 of syncope. Although the mean idioventricular rate of the nine patients in the narrow QRS group (39.7 per min) was significantly faster than that of the 14 patients in the wide QRS group (35.3 per min; P less than 0.05), the rate did not distinguish symptomatic patients within either the narrow or the wide QRS groups. His bundle studies of the site of block and the effect of single and paired right ventricular stimulation upon idioventricular rhythm were also unhelpful. The response of the return cycle to increasingly premature ventricular extrasystoles, however, proved more complex than was anticipated.

Thirty-three cases of congenital syphilis that were seen at The Children's Hospital, Camperdown, over a 34-year period were reviewed. Twenty-nine cases were examples of early congenital syphilis, as the patients developed clinical features in the first two years of life, and the other four cases were examples of late congenital syphilis, with the clinical features developing between two years and nine months of age and 10 years of age. Twenty-five patients were symptomatic at the time of admission to hospital. The most common clinical feature on the patient's presentation to hospital was a maculopapular or vesiculobullous skin eruption which occurred in 13 of the infants. Hepatic and splenic enlargement were present in 12 cases, and nine children had the "snuffles". Five of the children died. Patient follow-up occurred in fewer than 40% of cases and three of the children who were followed-up suffer from developmental delay. Congenital syphilis can occur in all social groups but is most common in infants who are premature or are small for gestational age and are born to young, unmarried mothers of low socioeconomic status. The treatment of congenital syphilis is simple and effective although the patients may need to be linked with community-health facilities to assist in their compliance with follow-up appointments.

In the pre-hospital phase of myocardial infarction, the overriding problem is our present inability to alter the fact that approximately 50 per cent of all deaths from infarction occur prior to the patient's arrival in hospital, despite the knowledge that these deaths are due largely to potentially correctible arrythmias.

Coronary angiography was performed and risk factors were evaluated 2-4 months after myocardial infarction in 50 men aged 40 years or less. There was a high incidence of heavy cigarette smoking, obesity and hyperlipidaemia among these young men. Single-vessel disease (greater than or equal to 70% coronary obstruction) was found in 52%, double-vessel disease in 22% and triple-vessel disease in 20%, the right coronary artery being more frequently involved (greater than or equal to 70% obstructed) or totally occluded than the left anterior descending or left circumflex coronary arteries, in that order. One of the 2 patients with a normal coronary arteriogram had left ventricular angiographic evidence of previous infarction. There was no apparent difference in the distribution of coronary artery disease in this group of young men from the reported distribution found in older subjects. Neither was there any significant correlation of any single major coronary risk factor or combination thereof with the extent or severity of coronary artery obstruction.

This report concerns a patient with complete heart block, in whom electrophysiological studies showed at times an escape rhythm with narrow QRS complexes preceded by His potentials with normal HV intervals (35--40 msec) and at other times an escape rhythm of similar rate, having wide QRS complexes of left bundle branch block configuration with no preceding His bundle activity. Complexes intermediate in width and configuration and preceded by His potentials with an HV interval inversely proportional to QRS width were also recorded. These observations are explained by a site of block proximal to the His bundle and competition between two pacemaker foci having similar discharge rates, one situated in the junctional region below the site of block and the other more distally in the right bundle branch or right ventricle. It is proposed that the combination of a proximal site of block and a distally situated dominant pacemaker may be a common reason for failure to record a His potential in patients with complete heart block.

Electrophysiological studies of atrioventricular conduction during rapid atrial overdrive pacing and during programmed premature atrial stimulation are reported in four patients with an unusually rapid 1:1 ventricular response to atrial flutter (ventricular rates 240 to 310 per minute). Second-degree AV block development during atrial overdrive pacing at rates well below those during spontaneous atrial flutter. Although none of the four patients showed evidence of pre-excitation on the standard 12-lead electrocardiogram, evidence suggesting a partial AV nodal bypass was demonstrated at electrophysiological study in one case. It is postulated that the profile of the atrial wavefront presented to the normal AV node by atrial flutter differs from that during high right atrial pacing and may account for the lower ventricular rates achieved during high right atrial overdrive pacing than during spontaneous atrial flutter in the remaining three cases.