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A mother with apparently balanced translocation between chromosomes 4 and 22 gave birth to two children (sib 1 and twin A) with 45,XX,der(4)t(4;22) (p16.3;q11.2)mat,-22 and 45,XY,der(4)t(4; 22(p16.3;q11.2)mat,-22 karyotypes. The mother was a slow learner and required special education. The imbalance in the sibs arose through a 3:1 malsegregation in the mother. The net result was deletions 4p16.3pter and 22q11.2pter. Deletion 4p is associated with Wolf-Hirschhorn syndrome (WHS). The 22q11.2 microdeletion is associated with a wide range of overlapping phenotypes including DiGeorge syndrome (DGS), velocardiofacial syndrome (VCFS), conotruncal facial abnormality, and sporadic or familial cardiac defect. Fluorescence in situ hybridisation (FISH) was performed. Cosmid probes D4S96, which maps to 4p16.3, and D22S75, which maps to 22q11.2, were used. In the mother, the translocation breakpoints were proximal to D4S96 on chromosome 4 and distal D22S75 on chromosome 22. The two sibs had deletions of a D4S96 and a D22S75 probe loci. Sib 1, a 2 1/2 year old girl, has multiple congenital abnormalities and profound developmental delay. The craniofacial features were generally of WHS. Hypoplasia of the thymus hypocalcaemia, and seizures in early infancy, which are clinical features of DGS, were also observed. Twin A was one of a pair of dizygotic twins. He had multiple congenital abnormalities and died soon after birth. [ABSTRACT FROM PUBLISHER]

Objectives: The objective of this study was to test the hypothesis that simulation training improves echocardiography skills., Study Design: In this prospective study, 43 participants (16 neonatologists, 26 neonatology fellows, and 1 nurse practitioner) were tested on the simulator after 4 hours of didactic sessions on topographical cardiac anatomy and standard echocardiographic views. Participants were given 20 minutes to obtain and save the standard views. After the simulation hands-on training for 8 hours over 3 days, they were tested again. Each image was scored from 0 to 3 based on the image quality. Pre- and postsimulator training data were compared using both automated and visual scoring methods., Results: After the hands-on simulator training, the automated median (interquartile) score for the quality of acquired images increased from 36 (22, 43) to 55 (48, 58), p  < 0.0001. The increase was similar using visual scoring. The number of views with acceptable or good image quality (scores of 2 or 3) increased from 11 (6, 16) to 20 (17, 21), p  < 0.0001. The neonatology fellows and faculty, as well as those with or without prior echocardiography experience, demonstrated significant improvement., Conclusions: Echocardiography simulation is an effective tool in improving echocardiography skills among neonatology care providers., Key Points: · Simulation is effective in acquiring echocardiography skills among neonatology care providers.. · Simulation improves image acquisition in those with and without prior echocardiography experience.. · Effectiveness of simulation in retaining the acquired echocardiography skill should be investigated.., Competing Interests: S.N., M.E., R.R., and B.S. have a financial interest in Virtual Echo Training System Inc., which has developed the neonatal echocardiography simulator., (Thieme. All rights reserved.)

Background: Foramen ovale (FO) is an obligate fetal shunt that typically resolves after birth, although patency throughout life is not uncommon. The natural history of patent FO (PFO) is known in term infants, but less is known about its course in extremely preterm infants. We describe the echocardiographic changes in FO size from birth to discharge in extremely low birth weight (ELBW) infants in this retrospective study., Methods: Cohort was divided based on size of FO at birth. Size of FO at discharge was measured and evaluated relative to postnatal weight gain. Demographics and clinical outcomes were compared between the two groups., Results: Of the 54 ELBW infants, 50 were born with FO less than 3 mm in diameter (small), and 4 were born with FO greater than 3 mm (large). Of small defects, the majority (44/50, 88%) did not increase in size with weight gain, and minority (6/50, 12%) increased in size, and three of these 6 patients, FO grew to be slightly larger than 3 mm. In contrast, all large defects (4 of 4, 100%) nearly doubled in size with postnatal growth. These 4 ELBW infants with enlargement of FO had a flap valve evident on echocardiogram obtained prior to discharge, and subsequently closed on outpatient follow-up echocardiograms, although time to resolution was variable (6 months - 3 years). One infant had presumptive resolution because of the presence of flap valve., Conclusion: No maternal or neonatal demographic characteristics were predictive of FO enlargement, although, demonstrable flap valve on discharge echocardiogram correlated with resolution of FO on outpatient follow-up echocardiogram. Therefore, based on our data, we recommend that ELBW infants born with large FO should have echocardiographic re-evaluation of the atrial septal opening prior to discharge, to specify the presence of a flap valve or lack thereof, which is an important detail that can help a neonatologist determine the need for outpatient cardiac follow-up., (© 2023. The Author(s).)

Objective: Pulmonary hemorrhage is a rare but severe complication of extremely low birth weight (ELBW) infants. The association of hemodynamically significant patent ductus arteriosus (hsPDA) and the diameter of the foramen ovale (FO) with pulmonary hemorrhage has not been reported., Study Design: Case control study of ELBW infants with and without pulmonary hemorrhage. Each ELBW infant with an echocardiogram within 48 h of pulmonary hemorrhage was analyzed., Result: 16 infants with pulmonary hemorrhage were matched with 32 controls by birth weight and gestational age. Echocardiogram showed hsPDA in all infants and those with pulmonary hemorrhage had significantly smaller patent FO [PFO] (1 vs 2.4 mm, p < 0.01) (OR 0.007; 95% CI 0.00007, 0.67 p = 0.03). Incidence of pulmonary hemorrhage was 8.9%., Conclusion: ELBW infants with hsPDA who experienced pulmonary hemorrhage had a significantly restricted or closed FO. Evaluation of FO should be considered with serial echocardiograms when evaluating for hsPDA., (© 2022. The Author(s), under exclusive licence to Springer Nature America, Inc.)

Point-of-care ultrasound (POCUS) refers to the use of portable ultrasound (US) applications at the bedside, performed directly by the treating physician, for either diagnostic or procedure guidance purposes. It is being rapidly adopted by traditionally non-imaging medical specialties across the globe. Recent international evidence-based guidelines on POCUS for critically ill neonates and children were issued by the POCUS Working Group of the European Society of Pediatric and Neonatal Intensive Care (ESPNIC). Currently there are no standardized national or international guidelines for its implementation into clinical practice or even the training curriculum to monitor quality assurance. Further, there are no definitions or methods of POCUS competency measurement across its varied clinical applications., Conclusion: The Hippocratic Oath suggests medical providers do no harm to their patients. In our continued quest to uphold this value, providers seeking solutions to clinical problems must often weigh the benefit of an intervention with the risk of harm to the patient. Technologies to guide diagnosis and medical management present unique considerations when assessing possible risk to the patient. Frequently risk extends beyond the patient and impacts providers and the institutions in which they practice. POCUS is an emerging technology increasingly incorporated in the care of children across varied clinical specialties. Concerns have been raised by clinical colleagues and regulatory agencies regarding appropriate POCUS use and oversight. We present a framework for assessing the risk of POCUS use in pediatrics and suggest methods of mitigating risk to optimize safety and outcomes for patients, providers, and institutions., What Is Known: • The use POCUS by traditionally non-imaging pediatric specialty physicians for both diagnostic and procedural guidance is rapidly increasing. • Although there are international guidelines for its indications, currently there is no standardized guidance on its implementation in clinical practice., What Is New: • Although standards for pediatric specialty-specific POCUS curriculum and training to competency have not been defined, POCUS is likely to be most successfully incorporated in clinical care when programmatic infrastructural elements are present. • Risk assessment is a forward-thinking process and requires an imprecise calculus that integrates considerations of the technology, the provider, and the context in which medical care is delivered. Medicolegal considerations vary across countries and frequently change, requiring providers and institutions to understand local regulatory requirements and legal frameworks to mitigate the potential risks of POCUS., (© 2021. The Author(s).)

Introduction: Simulators are increasingly used for training in echocardiography. However, there is no objective method to assess the skills acquired. Our objective was to develop and test an automated method to assess echocardiography skills., Methods: To automate the image quality evaluation, we expanded our previously developed neonatal echocardiography simulator to enable recording of images of the 26 standard cuts and process the image quality. We then compared the automated and visual methods in scoring image quality of the echocardiograms obtained by 22 trainees., Results: Each echocardiographic image representing a slice of a three-dimensional volume possesses 3 axes (X, Y, and Z) that correspond to the roll, pitch, and yaw angles of the transducer, respectively. Therefore, if the placement and orientation of the transducer are correct, the acquired image represents the appropriate cardiac window with the desired orientation in all 3 axes. The automated system gives a score of 0 if the transducer is not in the appropriate cardiac window. A score of 1, 2, or 3 is given if the image falls within the range of one, two, or three angles, respectively. There was no difference in the image quality score between automated and visual assessment methods (46.0 ± 13.0 vs 45.1 ± 14.4, P = .19). The two methods had excellent correlation (r = .95). The bias and precision were 0.9 and 8.8, respectively., Conclusions: The automated method is comparable to visual method for assessment of image quality. The automated process allows for instantaneous feedback and has the potential to standardize assessment of echocardiography skills of trainees., (© 2020 Wiley Periodicals LLC.)

Objective: This study aimed to determine the incidence of systemic to pulmonary collaterals (SPCs) in extremely low birth weight infants and to assess its clinical and hemodynamic significance beyond the neonatal period., Study Design: Retrospective cohort study was conducted on 61 infants with echocardiogram performed at the time of discharge to determine the presence of SPC and to measure the right and left ventricular outputs and left atrium to aortic ratio. We compared two groups: small or no SPC (Group 1) to moderate or large SPC (Group 2) on demographics, clinical outcomes, and echocardiographic parameters., Results: Sixty-one infants were included. The incidence of SPC was 57%; 21% of infants had moderate or large shunts and 31% had small SPC. Demographics, clinical outcomes, and echocardiographic parameters were not significantly different between small or no SPC and moderate to large SPC., Conclusion: More than half of the infants had SPC. The size of the shunt did not affect the clinical outcomes nor the echocardiographic parameters measured. All infants had cardiac output above the normative mean., Competing Interests: None declared., (Thieme. All rights reserved.)

We describe a case of an extremely low birthweight infant with a large patent ductus arteriosus and closed foramen ovale resulting in markedly increased pulmonary blood flow, pulmonary venous congestion, and pulmonary hemorrhage.

There is a great need for training in pediatric echocardiography. In addition to physicians being trained in pediatric cardiology and echocardiography technologists, neonatologist, pediatric intensivists, and other health care professionals may be interested in such training. Since, there is limited opportunity of training on live patients, echocardiographic simulators may be of help. No simulator with complete range of echocardiographic modalities is available for neonates and infants. The aim of this project was to develop a mannequin-based echocardiographic simulator capable of simulating full range of pediatric 2D, color flow Doppler, spectral Doppler, and M-mode echocardiograms. A mannequin, a laptop computer, a magnetic tracking device, and a six-degree freedom (6DOF) sensor incorporated in a dummy transducer serve as the hardware platform of the simulator. We obtained six to seven 4D echocardiographic datasets in DICOM format through five acoustic windows from each infant along with a complete set of 2D video clips of color flow, Doppler, and M-mode. The 4D datasets are sliced into 3D slices using the visualization toolkit and are displayed as 2D echocardiograms through the information obtained by the 6DOF sensor. The coordinates from specific 3D slices triggers display of video clips of color flow, M-mode, and Doppler echocardiogram. Software written in C++ programming language controls the basic function of the program. The main simulator screen displays the full range of 2D echocardiograms including color flow Doppler, spectral Doppler, and M-mode from each acoustic window, whereas the side screen display the position and motion of the cutting planes through a 3D heart model. The system includes a software module to perform hemodynamic measurements from specific video clips images. Our hybrid, mannequin-based pediatric echocardiography simulator provides full range of pediatric echocardiography training experience. This simulator may help training in pediatric echocardiography for which there is a growing demand in clinical medicine.

Objective: We sought to investigate whether the increase in blood pressure and decrease in vasopressor support after hydrocortisone administration are associated with changes in systemic hemodynamics in neonates who receive high-dosage dopamine to maintain blood pressure at the lowest acceptable levels., Methods: In this prospective, observational study, preterm and term neonates who required dopamine > or = 15 microg/kg per minute to maintain minimum acceptable blood pressure received intravenous hydrocortisone 2 mg/kg followed by up to 4 doses of 1 mg/kg every 12 hours. Fifteen preterm and 5 term neonates without a patent ductus arteriosus composed the study population. Echocardiograms and vascular Doppler studies were performed immediately before the first dose of hydrocortisone and at 1, 2, 6 to 12, 24, and 48 hours thereafter., Results: In the 15 preterm infants, during the first 12 hours of hydrocortisone treatment, the 28% increase in blood pressure paralleled that in the systemic vascular resistance without changes in stroke volume or cardiac output, whereas dopamine dosage decreased. By 24 hours, the dosage of dopamine continued to decrease, whereas stroke volume increased without additional changes in systemic vascular resistance. By 48 hours, dopamine dosage decreased by 72%; blood pressure and stroke volume increased by 31% and 33%, respectively; and systemic vascular resistance and cardiac output tended to be higher (14% and 21%, respectively) compared with baseline. Contractility, global myocardial function, and Doppler indices of blood flow in the middle cerebral and renal artery remained normal and unchanged. The findings in the 5 term infants showed a similar pattern for changes in cardiac function, systemic hemodynamics, and organ blood flow after hydrocortisone administration., Conclusions: In preterm and term neonates who require high-dosage dopamine to maintain blood pressure at the lowest acceptable levels, hydrocortisone improves blood pressure without compromising cardiac function, systemic perfusion, or cerebral and renal blood flow.

Background: Administration of hydrocortisone and relatively high doses of dexamethasone increase blood pressure in volume- and pressor-resistant hypotensive preterm infants. However, little is known about the temporal relationship of dexamethasone administration and the improvement in blood pressure and the weaning of pressors/inotropes. Furthermore, there are no sufficient data available on whether a smaller dose of dexamethasone would also be effective in treating refractory hypotension., Objective: To study the cardiovascular responses to low-dose dexamethasone in very low birth weight neonates with volume- and pressor-resistant hypotension., Methods: Retrospective database review. Twenty-four preterm neonates (gestational age 26 (23-34) weeks; birth weight 801 (457-1,180) g; postnatal age 2 (1-24) days, medians (ranges)) who remained hypotensive despite volume administration and combined dopamine and dobutamine treatment at >or=30 microg/kg/min received dexamethasone 0.1 mg/kg followed by 0.05 mg/kg intravenously every 12 h for 5 additional doses if still on pressors >or=8 microg/kg/min., Results: Two hours after the first dose of dexamethasone the mean blood pressure increased from 30 +/- 5 to 34 +/- 6 mm Hg (p < 0.001) and remained elevated at 4, 6, 12, and 24 h after treatment was started (p < 0.001). Six hours after the initial dose of dexamethasone the pressor/inotrope requirement decreased from 34 +/- 9 to 24 +/- 13 microg/kg/min (p = 0.001) and continued to decrease at 12 and 24 h (p < 0.001). Urine output also increased significantly during the first 6 h after dexamethasone (p < 0.001)., Conclusions: Low-dose dexamethasone rapidly increases blood pressure and decreases pressor requirements in very low birth weight neonates with volume- and pressor-resistant hypotension.

The aim of the study was to detect changes in pulmonary function following ligation of a patent ductus arteriosus (PDA). Pulmonary function was recorded in 16 newborns (birth weight 1,081 +/- 166 g, gestational age 27.6 +/- 1.7 weeks) before and after ligation. No change in resistance of airways or mean airway pressure was observed. We found an increase in dynamic compliance (Cdyn) of 77% (p < 0.01), in tidal volume (TV) of 29% (p = 0.004), and in minute ventilation (MV) of 17% (p < 0.01) after the procedure. We demonstrated that pulmonary function improves after surgical ligation of the PDA. Because of considerable variation in intubated and spontaneously breathing premature newborns, we recommend the analysis of three main parameters: Cdyn, TV and MV for estimation of pulmonary mechanics in these infants., (Copyright 2004 S. Karger AG, Basel)

We report a term infant with Pompe disease presenting in the immediate newborn period. The infant was born at 40 weeks' gestation, weighing 3600 g to a 32 year-old black female. Infant presented at delivery with massive hypertrophic cardiomyopathy and pulmonary hypertension. Diagnosis was confirmed by low alpha-glucosidase activity. The histopathology and electron microscopic findings were consistent with Pompe disease. This is the second reported case of Pompe disease presenting at delivery.

Objective: Angiographic visualization of systemic to pulmonary collaterals (SPC) has been documented in premature infants needing prolonged ventilatory support. Noninvasive identification of such communications in premature infants was reported recently. The purpose of this study was to describe: 1) incidence, 2) clinical findings and implications, and 3) short-term follow-up of SPC diagnosed by echocardiography in very low birth weight (VLBW) infants admitted to the neonatal intensive care unit., Methods: From December 1, 1994 to August 31, 1996, 196 infants with birth weight <1500 g were admitted to the neonatal intensive care unit; 133 of them received serial echocardiographic evaluations at 1 to 2 days, at 2 weeks, and at 1, 2, and 3 months of life. Follow-up echocardiograms were scheduled at 6 months and 1 year of age for patients with SPC persisting at 3 months of age., Results: SPC were demonstrated in 88 patients (66%) at 1 to 90 days of life (mean 28 days). In most cases, the SPC originated at the distal aortic arch or the proximal descending aorta. Ten patients (11%) were treated for congestive heart failure. The symptoms improved and anticongestive therapy was discontinued in 9. One patient with persistent congestive heart failure underwent therapeutic cardiac catheterization and 1 prominent SPC was embolized., Conclusions: The incidence of SPC in VLBW infants is much higher than previously reported. We postulate that SPC are bronchopulmonary communications that enlarge and/or proliferate in response to a given stimulus. These communications are associated with increased time on positive pressure ventilation and length of stay in the hospital. SPC may lead to pulmonary edema and should be searched for in VLBW infants with a more complicated course. Echocardiographic examination with color Doppler performed in premature infants to evaluate left to right shunts should include careful search for systemic to pulmonary collaterals.echocardiography, systemic to pulmonary collaterals, aortopulmonary collaterals, prematurity, pulmonary edema.

The purpose of this study is to determine the incidence of reverse aortic arch flow in newborn infants with pulmonary hypertension, and no other identifiable cause for flow reversal, being considered for extracorporeal membrane oxygenation (ECMO) 2) compare the left ventricular output, and 3) outcomes, of these infants, with those in the group of patients with normal arch flow. We reviewed the medical records and echocardiograms of all infants referred to our institution for extracorporeal membrane oxygenation support for pulmonary hypertension, between August 1994 and April 1996. Neonates with pulmonary hypertension and reverse aortic arch flow had significantly lower left ventricular output (p = 0.005), and had significantly higher mortality (p = 0.04), than those with normal aortic arch flow. They required significantly higher ventilatory support (p = 0.01) and tended to need more inotropic support. Reverse aortic arch flow in newborn infants with pulmonary hypertension, and no other cause for the reverse flow, indicates significantly decreased left ventricular output, and may be a marker for a more difficult clinical course and poor prognosis. In this group of patients with pulmonary hypertension, the survival was significantly higher in infants with no PDA or with pure left to right shunt across a PDA.

The objective of this study was to evaluate the clinical, radiological, and echocardiographic findings in 11 neonates with aneurysm of ductus arteriosus presented in our institutions between 1993 and 1996, and to postulate a new theory for the pathogenesis of this lesion. Medical records, radiographic studies, and echocardiograms were reviewed. All infants underwent follow-up echocardiograms every 2 to 3 days until the aneurysm spontaneously resolved or surgery was performed. The infants were predominantly term males; six had evidence of fetal distress, two were diagnosed prenatally by fetal echocardiogram, chest X ray evidenced mediastinal mass in six patients. The first echocardiogram showed structurally normal heart with an aneurysmal patent ductus arteriosus. In eight patients the aneurysm completely resolved by 5 to 10 days. One infant underwent surgical resection of the aneurysm after observation for 11 days with no change in size. Thrombosis of the aneurysm was noted in two patients; both underwent surgery. Increasing reports of ductal aneurysms in infants may reflect the availability of high-resolution echocardiography and more frequent use of echocardiography in the neonatal intensive care unit. Spontaneous resolution occurred in the majority of cases as in previous reports. We postulate that, at least in some cases, aneurysm of the ductus arteriosus is a congenital lesion that may represent poststenotic dilation of the ductus due to turbulent flow through a stenotic segment at its pulmonary artery end during fetal life. The presence of aneurysm of the ductus arteriosus should be excluded in selected cases of fetal distress, by fetal echocardiography.

With advances in neonatal intensive care survival of extremely low birth weight (< 1 kg) infants has increased significantly over the past decade. Dexamethasone is used increasingly for the prevention and treatment of chronic lung disease in these infants. The impact of dexamethasone therapy on the incidence or severity of retinopathy of prematurity (ROP) remains controversial. We conducted a retrospective study to evaluate the association between short-term dexamethasone treatment and severe ROP in extremely low birth weight infants. From October 1989 to December 1992, 309 very low birth weight infants were admitted to the neonatal intensive care unit. A total of 266 infants (86%) survived until hospital discharge. Of these, 90 weighed less than 1 kg. Thirty-eight of 90 infants received short-term dexamethasone therapy for chronic lung disease and the remaining 52 infants did not. Infants treated with dexamethasone and those not treated with dexamethasone were comparable in birth weight (820 vs 828 gm), gestational age (26.5 vs 26.9 weeks), inborn (11 vs 14), and occurrence of sepsis (13/38 vs 21/52). Infants treated with dexamethasone required longer periods of mechanical ventilation (44 +/- 23 vs 26 +/- 15 days, p < 0.001), had longer duration of supplemental oxygen (57 +/- 28 vs 29 +/- 23 days, p < 0.001), had higher incidence of patent ductus arteriosus (28/38 vs 18/52, p < 0.0003), and required surfactant therapy more often for respiratory distress syndrome (17/38 vs 11/52, p < 0.01), when compared with infants who did not receive dexamethasone. Severe ROP developed in 16 infants (stage III or higher); 12 of these were in the dexamethasone-treated group (p < 0.003). Thirteen infants required cryotherapy; nine were from the dexamethasone-treated group (p < 0.13). This study demonstrates an apparent association between the incidence of severe ROP and dexamethasone therapy. Prospective, randomized, controlled studies are needed to correct for differences in severity of cardiorespiratory illness to establish whether a causal role exists for steroid therapy in ROP. Until such studies are available, careful consideration must be given to indications, dosage, time of initiation, and duration of treatment with dexamethasone in extremely low birth weight infants.

One of the shortcomings of antepartum testing in the post-term pregnancy is that it does not identify the majority of fetuses who develop abnormal intrapartum fetal heart rate changes. The purpose of this study was to determine whether antenatal cardiovascular evaluation could aid in the identification of post-term fetuses at risk for intrapartum heart rate abnormalities. Seventy-five patients with a gestational age greater than 41 weeks underwent a non-stress test, amniotic fluid index and real-time assessment of the heart for the presence or absence of a pericardial effusion. M-mode measurements of the right ventricular inner dimension (RVID), left ventricular inner dimension (LVID), biventricular outer dimension (BVOD) and Doppler velocimetry of the umbilical artery (S/D) were performed. Group I (n = 32) had normal intrapartum heart rate tracings. Group II (n = 20) had abnormal intrapartum fetal heart rate tracings but did not undergo emergency delivery. Group III (n = 23) had abnormal intrapartum fetal heart rate tracings but underwent emergency delivery. When comparing Group I with Group II, the latter had significant differences for abnormal RVID, RVID/LVID ratio, and pericardial effusion. When comparing Groups I and III, there were significant differences for RVID, RVID/LVID ratio, pericardial effusion, BVOD, LVID and amniotic fluid index. Neither the non-stress test nor S/D predicted abnormal intrapartum fetal heart rate patterns. For prediction of abnormal intrapartum heart rate patterns, the sensitivities of the RVID (0.79), LVID (0.33), RVID/LVID ratio (0.72) and BVOD (0.63) were 1.7-4 times greater than the non-stress test (0.19) and the sensitivities of the RVID, RVID/LVID ratio and BVOD were 2 times greater than the amniotic fluid index (0.28). The positive (0.50-0.86) and negative (0.42-0.68) predictive values were similar for all groups. To predict emergency delivery associated with abnormal heart rate tracings, the sensitivities of the RVID (0.83), RVID/LVID ratio (0.70) and BVOD (0.65) were 2.5-3 times greater than the non-stress test (0.26) and 1.5 times greater than the amniotic fluid index (0.39). The positive (0.36-0.56) and negative (0.70-0.86) predictive values were similar. The presence of pericardial effusion had a higher sensitivity than the non-stress test and amniotic fluid index for predicting abnormal intrapartum heart rate patterns but not emergency delivery. Doppler velocimetry of the umbilical artery had a lower sensitivity than the non-stress test and amniotic fluid index for predicting intrapartum heart rate patterns as well as identifying the fetus needing emergency delivery. The results of this study would suggest that there is initially dilatation of the right ventricle which may be associated with abnormal intrapartum fetal heart rate patterns. However, when the left ventricle dilates, leading to cardiomegaly, there is a greater incidence of abnormal intrapartum fetal heart rate changes and associated emergency delivery. The amniotic fluid index appears to be a later finding for predicting abnormal intrapartum fetal heart rate changes., (Copyright 1991 International Society of Ultrasound in Obstetrics and Gynecology)

Sequential hemodynamic and biochemical changes were studied in 24 infants with sepsis due to beta-hemolytic streptococcus to define the temporal patterns of physiologic events and to compare them in surviving (n = 11) and nonsurviving (n = 13) infants. Septicemia was documented by positive blood culture in all. Biophysical and biochemical measurements were obtained before and hourly, for 11 h after antibiotic therapy was initiated. Surviving infants had significantly higher Hct and systolic and mean arterial pressures than nonsurvivors. In nonsurvivors, low BP was associated with a concomitant rise in CVP and severe metabolic acidosis refractory to therapy. Although there were no differences in PaO2 or PaCO2 between survivors and nonsurvivors, arterial-alveolar oxygen gradients were significantly greater in nonsurviving infants. These data show cardiorespiratory and metabolic alterations that differentiate surviving and nonsurviving infants with beta-hemolytic streptococcal septicemia.

M-mode echocardiographic examination of the right and left ventricles was performed on 82 normal fetuses between 18 and 41 weeks of gestation. The diameter of the right and left ventricles correlated with the biparietal diameter. The right/left ratio of the ventricular diameters remained constant (1:1) throughout gestation. Fractional shortening and mean circumferential shortening of both ventricles were independent of gestational age. The data presented in this paper will allow the clinician to evaluate abnormal fetal conditions that might affect cardiovascular structure and/or function.

The incidence of clinically detectable patient ductus arteriosus (PDA) in a group of preterm infants whose birth weights were less than 2,000 g was compared with that of a similar group of infants whose mothers received antenatal glucocorticoids. A PDA was diagnosed on the basis of a typical heart murmur, increased precordial activity, and bounding peripheral pulses beyond the third day of life. Whereas 14 (44%) of 32 infants who were not exposed to antenatal glucocorticoids showed evidence of a PDA, only one (6.5% of 15 infants whose mothers received antenatal glucocorticoids had similar findings. The incidence of ruptured membranes (greater than 72 hours), the number of infants who were small for gestational age, and clinical management of the infants in the two groups were similar.

Neonatal heart rate variability (NHRV) was studied in 92 preterm infants (birth weight, 750 to 2,500 gm; gestational age, 28 to 36 weeks). Each infant was monitored continuously during the first 6 hours and for one hour at 24, 48, and 168 hours of life. During each hour NHRV was quantified and related to the following parameters: sex, gestational age, postnatal age, heart rate, and the presence and severity of respiratory distress syndrome (RDS). NHRV in healthy preterm infants was inversely related to heart rate level and directly related to the infant's postnatal age. In healthy babies with gestations of 30 to 36 weeks there was no significant correlation between NHRV and gestation. Decrease in NHRV was significantly related to the severity of RDS, and the reappearance of NHRV in infants with RDS was associated with a good prognosis. Decreased NHRV significantly differentiated the infants with RDS who survived after the fifth hour of life. The data reveal that NHRV (1) should be corrected for heart rate level and postnatal age; (2) is decreased in RDS; and (3) can be used as an indicator of morbidity and mortality in preterm infants with RDS.

Ouabain-induced tachyarrhythmias in cats can be abolished by bolus injection of NaHCO3 solution. This effect is consistent, rapid and of short duration. The antiarrhythmic effect appears to be due to high sodium concentration of this solution, as hypertonic NaCl solution with equal concentration of sodium exerts the same effect. Alkalinity and hyper-osmolality cannot account for the antiarrhythmic effect of NaHCO3 as respiratory alkalosis and mannitol solution with equal osmolality did not relieve the arrhythmias. Overdrive suppression is not responsible for this effect as the heart rate slows down during normalization of the cardiac rhythm. It is postulated that high Na+ concentration abolished ouabain-induced arrhythmias by competition with calcium ion in the myocardial cell sarcotubular system thus replacing it. This hypothesis is supported by the known effect of other agents such as sodium-EDTA and magnesium chloride, in digitalis-induced arrhythmias, which exert their antiarrhythmic properties by removal or displacement of calcium in the myocardial cells.

VVLBW infants pose a challenge in the management of their hemodynamic changes. Data are just accumulating regarding the hemodynamic variables in this very special group. We have described several newly developed methods of monitoring hemodynamic variables in infants less than 1000 gm. In the coming decade, some of these methods may become more refined and routine.

Noninvasive monitoring of cardiac output can greatly facilitate the clinical assessment and management of neonates with cardiovascular compromise. To assess normal values of cardiac output in neonates, mean blood flow velocity was measured in the ascending aorta from a suprasternal approach using a range-gated, pulsed Doppler velocity meter, and aortic root diameter was determined from an M-mode echocardiogram. These techniques were combined, and cardiac output was evaluated in 59 healthy premature and 62 term newborn infants during the first week of life. Birth weights ranged from 780 g to 4,740 g and gestational age from 27 to 42 weeks. Cardiac output values increased linearly with advancing birth weight (r = +.94, P less than 0.001) and gestational age (r = +.95, P less than .001). Mean cardiac output values (+/- SD) per kilogram of body weight were 249 +/- 34 mL/min/kg and decreased with advancing birth weight: less than 1,500 g = 265 +/- 32 mL/min/kg; 1,500 to 2,500 g = 253 +/- 34 mL/min/kg; and greater than 2,500 g = 241 +/- 33 mL/min/kg. For clinical use, 325 mL/min/kg and 200 mL/min/kg can be used as upper and lower limits of normal, respectively. Doppler cardiac output estimates compared favorably with studies using invasive techniques.

A comparative morphometric study was performed on the aortomitral valve apparatus of the embryonic and adult chicken hearts. It was found that the thickness of the aortic and mitral valve cusps diminishes dramatically as the embryo matures, however the relative length of the aortic valve, mitral valve and subaortic conus remains constant. A comparison of the aortomitral valve apparatus in chicken and man revealed that the subaortic conus is not completely resorbed in the fowl, whereas this process is well documented in the mammalian hearts. The implications of this observation on "the differential conal growth hypothesis" of transposition of the great arteries are discussed.

During the period from February, 1971 to February, 1973, 30 premature infants underwent surgical ligation of patent ductus arteriosus. The gestational ages ranged from 25 to 36 wk (mean 30), and the birth weights ranged from 760 to 2010 g (mean 1274). The patients were divided into two groups on the basis of the indications for assisted ventilation. Group I consisted of 21 patients with severe hylanine membrane disease who required assisted ventilation during the first 2 days of life and could not be weaned off the respiratory by 10 days of age. Group II was composed of nine infants who required intermittent positive-pressure breathing after a mean age of 8 days because of repeated apneic spells secondary to uncontrollable heart failure. All infants in Group II survived the operation and left the hospital well. Fourteen of the 21 patients in Group I survived; the seven deaths were all due to underlying severe pulmonary disease (bronchopulmonary dysplasia). The value of PDA ligation in premature infants with uncontrollable heart failure is demonstrated in this study; this procedure also appears to be beneficial in neonates with severe respiratory distress syndrome.

Disopyramide has been successfully used to treat a variety of supraventricular and ventricular arrhythmias. The electrophysiologic effects of this drug were studied following diagnostic cardiac catheterization in 14 children aged 7 months to 14 years, suffering from congential heart disease. Surface EKG, intra-atrial, and His bundle electrogram as well as right atrial pacing with the use of extrastimulus technique were obtained before and after a single intravenous dose of disopyramide (2 mg/Kg, max 50 mg). The drug significantly prolonged the HV interval, effective and functional refractory periods of the atrium and the AV node. The most consistent change was the prolongation of the atrial refractory periods. The electrophysiologic effects of this drug on the conduction system have been variable and this variability has been attributed to the degree of vagal tone. The findings in this age group are similar to adults with simultaneous cholinergic blockade. This might be due to the direct depressant effects of disopyramide on the atrial and AV nodal tissue, and point to the potential usefulness of this drug in the pediatric age group.

Tetralogy of Fallot has increased in incidence during the past decade. Although abnormalities of the four-chamber screening examination of the fetal heart identifies structural anomalies associated with semilunar and atrioventricular valve hypoplasias, the anomalies associated with tetralogy of Fallot are not as readily identifiable from the four-chamber view alone. This study was designed to determine whether aortic root dilatation, commonly observed in the newborn with tetralogy of Fallot, is a marker for tetralogy of Fallot diagnosed in utero. In 45 normal (control) fetuses the biparietal diameter, head circumference, abdominal circumference, and femur length were measured and M-mode measurements were made of the biventricular outer dimension and aortic root dimension. The aortic root dimension from seven fetuses (18, 19, 20, 23, 32, 33, and 34 weeks' gestation) with tetralogy of Fallot was increased in dimension when compared with cardiac (biventricular outer dimension) and noncardiac biparietal diameter, head and abdomen circumferences, and femur length parameters. In conclusion, incorporation of aortic root imaging with the four-chamber view of the fetal heart during a routine screening examination allows for identification of fetuses with tetralogy of Fallot.

The cardiovascular effects of theophylline were studied in 11 clinically stable preterm infants. Theophylline was given as aminophylline using a loading dose of 6.8 mg/kg and a maintenance dose of 2 mg/kg every 8 hours intravenously. Cardiac output, stroke volume, and heart rate were measured using a combination of pulsed Doppler ultrasound and M-mode echocardiography. Compared with day 0, an increase was found in both cardiac output (P less than 0.01) and stroke volume (P less than 0.02) on days 1, 2, and 3. By day 7, stroke volume was comparable to pretreatment values, whereas cardiac output was still increased. Heart rate was augmented significantly (P less than 0.01) throughout the treatment period. Mean arterial blood pressure did not change. All but one of the neonates had serum theophylline concentrations between 6 and 13 mg/L. We conclude that both inotropic and chronotropic effects are evident during the first days of theophylline therapy. The metabolic cost of the increased cardiac output in the preterm infant with theophylline therapy deserves further attention.

Eighty-two normal fetuses were scanned from 18 to 41 weeks of gestation. The following M-mode measurements were correlated with the femur length: end-diastolic right ventricular (r = 0.93), left ventricular (r = 0.92), biventricular outer (r = 0.94), and biventricular inner (r = 0.94) dimensions; tricuspid (r = 0.93) and mitral (r = 0.92) valve opening excursion; right (r = 0.76) and left (r = 0.70) ventricular wall thickness; and interventricular septal thickness (r = 0.75). Polynomial regression demonstrated that linear regression best described the data from which the mean and 5% and 95% confidence limits for predicted M-mode measurements were computed for the femur length.

Decreased cardiac output is a common presumption in left ventricular myocardial dysfunction in neonates, but because of a lack of reliable noninvasive techniques, data on cardiac output are missing. We measured cardiac output by pulsed Doppler echocardiography in 22 newborn infants with left ventricular myocardial dysfunction diagnosed by M-mode echocardiography. Eleven neonates had severe perinatal asphyxia, seven had tachypnea, two hypoglycemia, and one septic shock; one had no symptoms. Right ventricular function was abnormal in 13 of the 22 infants. Hypotension was found in eight; cardiac output and stroke volume were low in 20. The abnormalities were more pronounced in infants with asphyxia. Six such infants were given dopamine (4 to 10 micrograms/kg/min). Within 1 hour, arterial blood pressure, cardiac output, stroke volume, and heart rate increased sharply, with normalization of the myocardial contractility; the other echocardiographic abnormalities normalized over 24 to 48 hours. Pulsed Doppler echocardiography is an advance in the detection and evaluation of therapy for left ventricular myocardial dysfunction in the neonate.