Interaction between pulmonary vasculature and the patent ductus arteriosus in very premature infants.

BACKGROUND: The benefits of closing the ductus arteriosus in very preterm infants have not been convincingly shown in numerous clinical trials. Because a large untreated ductus arteriosus can cause death from congestive heart failure in infants born at term, we need to explain why this might not occur in premature infants born at <28 weeks' gestation. METHODS: Based on information in the literature, I have commented on the possible relationship between the pulmonary vasculature and the shunt through the patent ductus arteriosus. RESULTS: Many of these infants have bronchopulmonary dysplasia, in which animal and human studies have shown a reduced number of capillaries and small pulmonary arteries as well as reduction in vascular endothelial growth factor (VEGF) and platelet endothelial cell adhesion molecule-1 (PECAM-1). Both of these import angiogenic factors. Some who do not have bronchopulmonary dysplasia may have a restricted pulmonary vascular bed. CONCLUSIONS: The increased pulmonary vascular resistance in very premature infants may restrict pulmonary blood flow even if the ductus is large, thus reducing the urgency for ductus closure.

Pulse oximetry in neonates at high altitudes: a modified Colorado protocol.

Pulse oximetry for detecting critical CHD produces more false positive tests at high altitudes than at sea level, because at altitude the average resting saturation is lower and the variability is higher. This increases diagnostic difficulties, especially in small isolated communities without paediatric echocardio-graphy, and requires expensive transport to a regional medical centre. One way of reducing diagnostic errors is to measure arterial oxygen saturation while the infant is breathing 100% oxygen. In the absence of right-to-left shunting through the heart, the ductus, or the lungs, arterial oxygen tension will exceed 150 mmHg and arterial oxygen saturation will be 100%. With right-to-left shunting, arterial oxygen tension will be <100 mmHg, and thus <96% (usually much lower).

A Preliminary Study of Left Ventricular Rotational Mechanics in Children with Noncompaction Cardiomyopathy: Do They Influence Ventricular Function?

BACKGROUND: Current diagnostic criteria for noncompaction cardiomyopathy (NCC) lack specificity, and the disease lacks prognostic indicators. Reverse apical rotation (RAR) with abnormal rotation of the cardiac apex in the same clockwise direction as the base has been described in adults with NCC. The aim of this study was to test the hypothesis that RAR might differentiate between symptomatic NCC and benign hypertrabeculations and might be associated with ventricular dysfunction. METHODS: Echocardiograms from 28 children with NCC without cardiac malformations were prospectively compared with those from 29 age-matched normal control subjects. A chart review was performed to identify the patients' histories and clinical characteristics. Speckle-tracking was used to measure longitudinal strain, circumferential strain, and rotation. RESULTS: RAR occurred in 39% of patients with NCC. History of left ventricular (LV) dysfunction or arrhythmia was universal in, but not exclusive to, patients with RAR. Patients with RAR had lower LV longitudinal strain but similar ejection fractions compared with patients without RAR (median, -15.6% [interquartile range, -12.9% to -19.3%] vs -19% [interquartile range, -14.5% to -21.9%], P < .01; 53% [interquartile range, 43% to 68%] vs 61% [interquartile range, 58% to 67%], P = .08). Only a pattern of contraction with RAR, early arrest of twisting by mid-systole, and premature untwisting was associated with lower ejection fraction (46%; interquartile range, 43% to 52%; P = .006). CONCLUSIONS: RAR is not a sensitive but is a specific indicator of complications in children with NCC. Therefore, RAR may have prognostic rather than diagnostic value. Premature untwisting of the left ventricle during ejection may be an even more worrisome indicator of LV dysfunction.

The Ratio Fallacy, with Special Reference to the Cardiac Index.

When comparing cardiac outputs in children of different sizes, or deciding on the normality of these outputs, we often convert the absolute output to the output per m2 body surface area-the cardiac index. For small infants, this leads to potentially large errors. The best way to evaluate these outputs is to determine their z values.

Will the real ventricular architecture please stand up?

Ventricular twisting, essential for cardiac function, is attributed to the contraction of myocardial helical fibers. The exact relationship between ventricular anatomy and function remains to be determined, but one commonly used explanatory model is the helical ventricular myocardial band (HVMB) model of Torrent-Guasp. This model has been successful in explaining many aspects of ventricular function, (Torrent-Guasp et al. Eur. J. Cardiothorac. Surg., 25, 376, 2004; Buckberg et al. Eur. J. Cardiothorac. Surg., 47, 587, 2015; Buckberg et al. Eur. J. Cardiothorac. Surg. 47, 778, 2015) but the model ignores important aspects of ventricular anatomy and should probably be replaced. The purpose of this review is to compare the HVMB model with a different model (nested layers). A complication when interpreting experimental observations that relate anatomy to function is that, in the myocardium, shortening does not always imply activation and lengthening does not always imply inactivation.

The history of the microsphere method for measuring blood flows with special reference to myocardial blood flow: a personal memoir.

We use many types of equipment and technologies to make our measurements but give little thought to how they developed. Evolution was once described as a series of recoils from blind alleys, and this is exemplified by the gradual development of the microsphere method of measuring blood flows. The microsphere method is one of the most frequently used methods for measuring blood flow to organs and portions of organs. The method can measure myocardial blood flow with reasonable accuracy (within 10%) down to samples weighing >50 mg but probably will not do so for samples weighing 1-10 mg. Microspheres with diameters from 10 to 15 µm provide the best compromise between accurate flow measurement and retention in tissue. Radioactive labels have been almst entirely replaced by fluorescent labels, but colored microspheres and neutron-activated labels are also used.NEW & NOTEWORTHY The contributions of the various individuals who developed the microsphere method of measuring regional blood flows and how these advances took place are brought to light in this paper.

At what age should tetralogy of Fallot be corrected?

Tetralogy of Fallot can be corrected with very low mortality at any age, even in neonates, but this does not necessarily mean that it should be corrected in the neonatal period. Although there are many advantages to early correction, a high proportion of these neonates have residual stenosis or pulmonary regurgitation that impairs ventricular function and may require further surgery or implantation of a pulmonary valve. Before we had the ability to correct this anomaly with low mortality in small children, a variety of palliative procedures had to be performed. Today, with better understanding of the anatomy of tetralogy of Fallot, we should consider what forms of palliation will increase growth of the right ventricular outflow tract in order to reduce the complications of very early surgery.

Is spontaneous closure of a patent arterial duct common?

As closing a patent arterial duct is relatively simple, safe, and successful, most children with a patent arterial duct have it closed soon after diagnosis. The larger ducts are closed to prevent congestive heart failure, pulmonary vascular disease, or aneurysmal dilatation of the ductus, and smaller ducts are closed to prevent infective endocarditis. Consequently, there is no opportunity to determine whether spontaneous closure or diminution in size of the patent arterial duct is common. If the duct does become smaller, flow through it may be so low that no murmur is produced - the silent ductus. The frequency and best management of the silent patent arterial duct are unknown, and we do not know whether these tiny ducts are the last stage before spontaneous closure.

Is Pulse Oximetry Useful for Screening Neonates for Critical Congenital Heart Disease at High Altitudes?

Now that pulse oximetry is used widely to screen for critical congenital heart disease, it is time to consider whether this screening method is applicable to those who live at high altitudes. Consideration of basic physical principles and reports from the literature indicate that not only is the 95 % cutoff point for arterial oxygen saturation incorrect at high altitudes, but the lower saturations are accompanied by greater variability and therefore there is the possibility of a greater percentage of false-positive screening tests at high altitudes. Because of ethnic differences in response to high altitudes, normative data will have to be collected separately in different countries and perhaps for different ethnic groups.

Ventricular structure-function relations in health and disease: part II. Clinical considerations.

Normal cardiac function of the left and right ventricles, together with the septum, is related to form/function interactions within the helical ventricular myocardial band. This knowledge is a prerequisite to understanding form/function interactions in diseases and for planning new treatments. Topics discussed include congestive heart failure in dilated hearts of ischaemic, valvar or nonischaemic origin as well as diastolic dysfunction. Similar thinking underlies novel treatments for dyssynchrony in pacing, together with focusing upon varying global left or right ventricular anatomy to correct mitral and tricuspid insufficiency caused by tethering of the leaflets. The septum is the lion of the right ventricle and insight is provided into offsetting septal damage during cardiac surgery, rebuilding its anatomical structure in post-tetralogy pulmonary insufficiency, as well as rectifying its dysfunction by decompression in patients with a left ventricular assist device.

Ventricular structure-function relations in health and disease: Part I. The normal heart.

The heart's structure-function relationships explain normal cardiac dynamics and clarify how they are disrupted by disease. For 500 years, anatomists described circumferential and helical cardiac fibres, yet disagreed about their relationships. One current model is attributed to Torrent Guasp who described functional pathways, the helical ventricular myocardial band (HVMB) with two interconnected loops: an outer basal loop with transverse fibres surrounds an inner apical helical loop that is composed of oblique descending and ascending segments that create a conical apical vortex. This review addresses the potential role of the HVMB in explaining the mechanics of isovolumic contraction, ejection, post-ejection isovolumic interval, rapid filling, torsion and recoiling. During the post-ejection isovolumic interval, a ∼ 90-ms hiatus exists between the end of contraction of the descending and the ascending segments. Compromise of this hiatus by disease disturbs the interdependence between torsion and 'untwisting' and impairs cardiac function. The validity of conventional expressions such as isovolumic relaxation, hyperechogenic septal line, untwisting and mitral valve opening will be revisited.

Right ventricular architecture responsible for mechanical performance: unifying role of ventricular septum.

The right ventricle (RV) is composed of a free wall containing a wrap-around circumferential muscle at its base and a septum composed of helical fibers that are oblique and cross each other at 60° angles. This structure is defined by the helical ventricular myocardial band and defines RV function because the wrap-around transverse fibers constrict or compress to cause the bellows motion responsible for 20% of RV output, whereas the oblique fibers determine shortening and lengthening that produces 80% of RV systolic function. Clinical shortening is quantified by tricuspid annular plane systolic excursion and measured by echocardiography. Destruction of the free wall by electrocautery or patch replacement does not alter RV function if the septum is intact. Conversely, septal damage causes RV dysfunction if pulmonary vascular resistance is increased. The interaction between structure and function to cause RV failure and how these factors become corrected is defined for RV failure, RV relationship to LV failure, resynchronization, pacing, RV dysplasia, left ventricular assist device, intraoperative septal injury during myocardial protection, the septal role in tricuspid insufficiency, pharmacologic decisions on altering pulmonary vascular resistance in RV failure, congenital heart disease, and adult heart disease is considered in this overview. These structure-function relationships emphasize why clinical decisions must be based on knowledge of normality, recognizing how disease offsets normality, and introducing actions that rebuild normality.

Abnormal origins of the coronary arteries from the aortic root.

Anomalous origin of a coronary artery from the aorta is a potentially serious anomaly that occurs in about 0.1-0.2% of the population. This percentage is small; however, it translates into about 4000 annual births with these anomalies. The clinical presentation of these anomalies is rare, and hence most are and will remain asymptomatic. The various anatomic anomalies are described, with anomalous origin of the left coronary artery that then passes between the aorta and pulmonary artery being the most serious of these anomalies. The pathophysiology resulting from these anomalies is described, as are methods for identifying those who require treatment; however, we still do not know the best methods of determining which patients need treatment.