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OBJECTIVE: To evaluate the growth of the pulmonary arteries after a Fontan procedure. DESIGN: Retrospective review. SETTING: Two paediatric cardiology tertiary care centres. PATIENTS: 61 children who underwent a modified Fontan operation and had angiography suitable for assessment of pulmonary artery size before the Fontan procedure and during long term follow up. An atriopulmonary connection (APC) was present in 23 patients (37.7%) and a total cavopulmonary connection (TCPC) was present in 38 (62.3%). Postoperative angiograms were performed 0.5-121 months (median 19 months) after the Fontan operation. MAIN OUTCOME MEASURE: Growth of each pulmonary artery measured just before the first branching point. The diameter was expressed as a z score with established nomograms used to standardise for body surface area. RESULTS: The mean change in the preoperative to postoperative z scores of the right pulmonary artery was -1.06 (p = 0.004). The mean change in the preoperative to postoperative z scores of the left pulmonary artery was -0.88 (p = 0.003). Changes in the preoperative to postoperative z scores were more pronounced in the patients undergoing APC than TCPC, especially for the right pulmonary artery. CONCLUSION: After the Fontan operation, growth of the pulmonary arteries often fails to match the increase in body surface area. [ABSTRACT FROM AUTHOR]

Background: Recurrent laryngeal nerve injury leading to vocal cord paralysis is a known complication of cardiothoracic surgery. Its occurrence during interventional catheterisation procedures has been documented in case reports, but there have been no studies to determine an incidence., Objective: To establish the incidence of left recurrent laryngeal nerve injury leading to vocal cord paralysis after left pulmonary artery stenting, patent ductus arteriosus device closure and the combination of the procedures either consecutively or simultaneously., Methods: Members of the Congenital Cardiovascular Interventional Study Consortium were asked to perform a retrospective analysis to identify cases of recurrent laryngeal nerve injury after the aforementioned procedures. Twelve institutions participated in the analysis. They also contributed the total number of each procedure performed at their respective institutions for statistical purposes., Results: Of the 1337 patients who underwent left pulmonary artery stent placement, six patients (0.45%) had confirmed vocal cord paralysis. 4001 patients underwent patent ductus arteriosus device closure, and two patients (0.05%) developed left vocal cord paralysis. Patients who underwent both left pulmonary artery stent placement and patent ductus arteriosus device closure had the highest incidence of vocal cord paralysis which occurred in 4 of the 26 patients (15.4%). Overall, 92% of affected patients in our study population had resolution of symptoms., Conclusion: Recurrent laryngeal nerve injury is a rare complication of left pulmonary artery stent placement or patent ductus arteriosus device closure. However, the incidence is highest in patients undergoing both procedures either consecutively or simultaneously. Additional research is necessary to determine contributing factors that might reduce the risk of recurrent laryngeal nerve injury.

Coastal sediments are rich in conductive particles, possibly affecting microbial processes for which acetate is a central intermediate. In the methanogenic zone, acetate is consumed by methanogens and/or syntrophic acetate-oxidizing (SAO) consortia. SAO consortia live under extreme thermodynamic pressure, and their survival depends on successful partnership. Here, we demonstrate that conductive particles enable the partnership between SAO bacteria (i.e., Geobacter spp.) and methanogens ( Methanosarcina spp.) from the coastal sediments of the Bothnian Bay of the Baltic Sea. Baltic methanogenic sediments were rich in conductive minerals, had an apparent isotopic fractionation characteristic of CO 2 -reductive methanogenesis, and were inhabited by Geobacter and Methanosarcina As long as conductive particles were delivered, Geobacter and Methanosarcina persisted, whereas exclusion of conductive particles led to the extinction of Geobacter Baltic Geobacter did not establish a direct electric contact with Methanosarcina , necessitating conductive particles as electrical conduits. Within SAO consortia, Geobacter was an efficient [ 13 C]acetate utilizer, accounting for 82% of the assimilation and 27% of the breakdown of acetate. Geobacter benefits from the association with the methanogen, because in the absence of an electron acceptor it can use Methanosarcina as a terminal electron sink. Consequently, inhibition of methanogenesis constrained the SAO activity of Geobacter as well. A potential benefit for Methanosarcina partnering with Geobacter is that together they competitively exclude acetoclastic methanogens like Methanothrix from an environment rich in conductive particles. Conductive particle-mediated SAO could explain the abundance of acetate oxidizers like Geobacter in the methanogenic zone of sediments where no electron acceptors other than CO 2 are available. IMPORTANCE Acetate-oxidizing bacteria are known to thrive in mutualistic consortia in which H 2 or formate is shuttled to a methane-producing Archaea partner. Here, we discovered that such bacteria could instead transfer electrons via conductive minerals. Mineral SAO (syntrophic acetate oxidation) could be a vital pathway for CO 2 -reductive methanogenesis in the environment, especially in sediments rich in conductive minerals. Mineral-facilitated SAO is therefore of potential importance for both iron and methane cycles in sediments and soils. Additionally, our observations imply that agricultural runoff or amendments with conductive chars could trigger a significant increase in methane emissions., (Copyright © 2018 Rotaru et al.)

ANaerobic MEthanotrophic (ANME) archaea remove the greenhouse gas methane from anoxic environments and diminish its flux to the atmosphere. High methane removal efficiencies are well documented in marine environments, whereas anaerobic oxidation of methane (AOM) was only recently indicated as an important methane sink in freshwater systems. Freshwater AOM-mediating microorganisms lack taxonomic identification and only little is known about metabolic adaptions to prevailing biogeochemical conditions. One of the first study sites providing information about AOM activity in freshwater sediment is Lake Ørn, a low-sulfate, iron-rich Danish lake. With the aim to identify freshwater AOM-mediating archaea, we incubated AOM-active anoxic, nitrate-free freshwater sediment from Lake Ørn with 13 C-labeled methane ( 13 C CH4 ) and 13 C-labeled bicarbonate ( 13 C DIC ) and followed the assimilation of 13 C into RNA by stable isotope probing. While AOM was active, 13 C CH4 and probably also 13 C DIC were incorporated into uncultured archaea of the Methanosarcinales -related cluster ANME-2d, whereas other known ANME lineages were not detected. This finding strongly suggests that ANME-2d archaea perform AOM coupled to sulfate and/or iron reduction and may have the capability of mixed assimilation of CH 4 and DIC. ANME-2d archaea may thus play an important role in controlling methane emissions from nitrate-depleted and low-sulfate freshwater systems.

Objective: Utilization of continuous transesophageal echocardiographic guidance (cTEE) during transcarotid balloon valvuloplasty (TCBV) in neonates and small infants with critical aortic valve stenosis (AS) allows for continuous hemodynamic assessment and improved outcomes., Background: Preferred method of intervention for critical AS remains controversial due to conflicting results., Methods: Since 1992, 30 neonates and small infants with critical AS and adequate left ventricular (LV) volumes underwent TCBV with cTEE. Critical AS was defined as ductal dependent systemic circulation, LV systolic dysfunction, or an echo gradient≥100 mm Hg with evidence of hypoperfusion., Results: The median age at intervention was 4 days (range 1-54 days). Nineteen (63%) patients required PGE1 and 25 (85%) had LV dysfunction. All procedures were performed with cTEE guidance. The initial 15 patients were performed in the cath lab whereas the subsequent 15 patients were performed at the bedside without fluoroscopy. The peak systolic gradient decreased from 70 to 24 mm Hg (P<0.001). Four (13%) early deaths were secondary to associated cardiac anomalies although one patient developed severe aortic valve insufficiency (AI) immediately post intervention. At discharge, two patients (8%) had ≥moderate AI. At a mean follow-up of 9 years (range: 2.2-20 years), there were 15 additional aortic valve interventions. Freedom from aortic valve reintervention at 10 years was 55% and actuarial survival rate at 10 and 15 years was 82%., Conclusion: Bedsides TCBV with cTEE guidance is effective palliation for neonates and small infants with critical AS and allows for continuous hemodynamic assessment without the use of ionizing radiation. Our early and late results appear comparable to surgical valvotomy., (© 2015 Wiley Periodicals, Inc.)

We describe the use of multiple Amplatzer vascular plugs which were intentionally placed so as to isolate a large azygous vein aneurysm with intraluminal thrombosis, which had resulted in a previous pulmonary embolus. This technique avoided the need for an invasive thoracotomy and aneurysm resection or attempting to directly fill the large aneurysm with numerous and various embolization devices., (Copyright © 2010 Wiley-Liss, Inc.)

Objectives: There is little evidence in the medical literature about safety of PDA device closure and its effects on aortic root diameter in Marfan patients. We reported on nine Marfan patients whose ducts were occluded by coil or Amplatzer Duct Occluder., Patients and Interventions: Two patients had aneurismal type E ducts which were closed by a single coil, respectively. The others had conical ducts for which even Amplatzer occluder, stiff, or double coils were used., Results: No untoward consequence was found during a median follow up period of 18.5 months (range 3 months to 6.33 years). Two patients had small residual shunts. The observed change in the mean ratio of aortic root diameter to its estimated value was statistically insignificant in seven patients who had complete data on their aortic root diameter before PDA closure and during the follow up., Conclusions: We concluded that PDA device closure in Marfan patients is safe. Risk of residual shunt is higher even after closure of small ducts.

A large secundum atrial septal defect in a 4-year-old child was closed by percutaneous placement of an Amplatzer septal occluder (AGA Medical Corporation, Plymouth, MN). After device placement, complete heart block developed that did not resolve after 3 days of medical management. The patient subsequently underwent surgical removal of the device and suture closure of the atrial septal defect. The patient recovered conduction after the operation. We report the successful treatment of device-induced heart block with surgical removal of the device., (Copyright (c) 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Reports about pacemaker (PM) dysfunction during irradiation (IR) are very rare, which is because of the extensive protective mechanisms that exist in these devices against electromagnetic interference (EMI). We report a case in which one of the most clinically relevant type of PM malfunctions, a runaway PM, occurred during radiation in a 76-year-old woman who was treated for inoperable esophageal cancer with a course of photon IR. The estimated IR dose of 0.11 Gy was the lowest in vivo dose ever reported. So a direct radiation effect as cause for this malfunction appears to be improbable. It could be concluded that the PM dysfunction was most likely induced by EMI during radiotherapy. The real reason of the device's software failure remains unclear.

We determined the utility of continuous wave (CW) Doppler for quantification of pulmonary insufficiency (PI) confirmed by pulmonary angiography in patients with postoperative adult congenital heart disease. A total of 41 patients with PI were divided into two groups on the basis of PI severity by pulmonary angiography: group A (n = 27) with severe PI and group B (n = 14) with mild or moderate PI. Nine patients in group A had pulmonic valve replacement and reverted to mild PI after surgery. Their pre- and postoperative data were compared. All underwent a two-dimensional/Doppler study with interrogation of the PI jet for jet width by color Doppler and peak flow velocity, deceleration time (DT), pressure half-time (PHT), diastolic period (DP), and PI flow time (FT) by CW Doppler. The no-flow time (NFT), NFT/FT ratio, and NFT/DP fraction were calculated. Group A had a larger right ventricle (4.1 +/- 0.9 vs. 3.5 +/- 0.6 cm, P = .033), higher PI peak velocity (2.1 +/- 0.5 vs. 1.7 +/- 0.5 m/s, P = .04), shorter DT (261 +/- 61 vs. 317 +/- 83 ms, P = .018) and PHT (76 +/- 29 vs. 132 +/- 53, P < .0001), longer NFT (146 +/- 66 vs. 40 +/- 42 ms, P < .0001), and higher ratios of NFT/FT (46% +/- 27% vs. 13% +/- 14%, P < .0001) and NFT/DP (29% +/- 13% vs. 10% +/- 9%, P < .0001). The PHT and DT lengthened, and the NFT shortened in patients who underwent pulmonic valve replacement (all P < .05). By binary logistic regression, NFT and PHT were the best predictors for severe PI. An NFT of 80 ms had 84% sensitivity and 93% specificity, and a PHT of 100 ms had 93% sensitivity and 93% specificity for identifying angiographically severe PI. CW Doppler accurately distinguishes severe from lesser degrees of PI in patients with postoperative adult congenital heart disease.

Objective: Assess the early and intermediate results with respect to blood pressure control in older children undergoing endovascular stenting for native coarctation of the aorta., Design: Eleven hypertensive patients (10 +/- 3 years of age) underwent endovascular stenting via standard techniques for native coarctation of the aorta as an alternative to surgical repair. Resting and exercise assessment of blood pressure control with Doppler echocardiography was performed pre- and poststenting., Results: All 11 patients underwent successful stenting without complications and were able to be weaned off antihypertensive medications within a short period of time. At latest follow-up (34 +/- 26 months), all patients are normotensive at rest and during treadmill exercise stress testing. Two patients underwent uncomplicated additional stent dilation at follow-up cardiac catheterization., Conclusion: Endovascular stenting for native coarctation of the aorta in older children is a reasonable alternative to surgical correction. During early follow-up, stenting effectively alleviates the aortic arch obstruction with normalization of the systemic blood pressure both at rest and during maximal exercise.

30 years ago the observation of a lower incidence of cardiovascular diseases in Inuits (Eskimos) was related to the higher fish consumption when compared to the residual Danish population. Clinical studies confirmed this finding. It was explained by the higher content of polyunsaturated fatty acids (PUFA) in fish, especially of omega-3 PUFAs. Experimental studies in cell cultures and also in animals with and without infarction models verified the anti-arrhythmic effect of omega-3 PUFAs among other possible contributing factors when compared to other fatty acids. In clinical studies a significant reduction (ca. 40%) of sudden cardiac deaths (SCD) could be found in patients after an acute myocardial infarction, if they were treated with at least 1 g omega-3 PUFAs daily, either by consumption of fish twice weekly or of a highly purified preparation omega-3 PUFAs in capsules. These findings led to recommendations of the American Heart Association and the European Society of Cardiology to a higher fish consumption and/or the daily intake of 1 g omega-3 PUFAs for primary and especially for secondary prevention of cardiovascular diseases. The much fewer side-effects, and the standardised dosage on one hand and the negative effect of the sometimes higher mercury content of fish make the intake of omega-3 PUFAs as capsules the better choice.

Therapy for severe aortic valve stenosis in infants and children has shifted from the operating suite to the catheterization laboratory and even to the bedside as a direct result of improved catheter technology, evolving techniques, and comparable results to conventional surgical intervention. This review summarizes the brief history pertaining to the various techniques and outcomes of transcatheter balloon valvuloplasty in infants and children with critical or severe aortic valve stenosis., (Copyright 2006 Wiley-Liss., Inc.)

I have previously reported the successful use of the 0.9 millimetre excimer laser catheter in 2 neonates with pulmonary valvar atresia and intact ventricular septum. In this report, I describe the expanded role of the excimer laser in a premature infant weighing 1.8 kilograms who presented with an occluded right pulmonary artery secondary to an organized thrombus. Successful reconstruction of the right pulmonary artery was performed by sequential use of lasers of 0.9, 1.4, and 2 millimetres diameter, followed by conventional balloon angioplasty. As is now the situation in adults, laser catheter intervention should now be an integral part of the armamentarium of the paediatric cardiac interventionalist.

Biventricular hypertrophy was noted at 24 weeks' gestation in a fetus with isolated cytochrome-c oxidase (COX) deficiency. Shock, caused by hypertrophic cardiomyopathy and severe pulmonary hypertension, led to the patient's death on day 6. His phenotype defines a new lethal variant of COX deficiency characterized by prenatal-onset cardiopulmonary pathophysiology.

Critical pulmonary valve stenosis or atresia with intact ventricular septum is a rare congenital cardiac defect that can be technically difficult to alleviate in the catheterization laboratory. Over the past 10 years, several techniques and modifications with variable results have been advocated to facilitate the valvuloplasty procedure. This report describes a single operator's experience using various techniques in 28 neonates with critical pulmonary stenosis or atresia who were considered candidates for transcatheter intervention. The first two patients underwent a gradational balloon valvuloplasty approach that resulted in prolonged fluoroscopy exposure. Thereafter, a "snare assisted" umbilical artery approach was developed which facilitated the valvuloplasty procedure and resulted in significantly fewer balloons used and shorter fluoroscopy times. Early in our experience, stiff guidewire perforation of atretic pulmonary valves was used, whereas in our last two patients, a simplified perforation technique with a new 0.9-mm excimer laser catheter was used. Late echocardiographic and clinical follow-up evaluation in 27 patients demonstrates persistent gradient relief, resolution of tricuspid valve insufficiency, and elimination of right to left shunting at the atrial level. Balloon valvuloplasty is the treatment of choice for critical pulmonary valve stenosis or atresia with intact ventricular septum. When necessary, the use of umbilical artery "snare assistance" facilitates the valvuloplasty technique and shortens procedure time while laser perforation is currently preferable for perforation of the atretic pulmonary valve., (Copyright 2002 Wiley-Liss, Inc.)

The occurrence of supradiaphragmatic decompressing venous collateral channels following construction of a bidirectional cavopulmonary connection or completion of the Fontan operation resulting in abnormal systemic hypoxemia has been infrequently described. In addition, the incidence and predictors of these channels have not been well delineated, especially in those patients without formation of such structures preoperatively. I evaluated, retrospectively, 40 patients who had undergone either construction of a bidirectional cavopulmonary shunt or completion of the Fontan operation, and who had complete pre and postoperative hemodynamic and angiographic data. Of the patients, 17 (43%) had developed a total of 21 decompressing venous collateral channels, of which 7 (18%) were considered to be hemodynamically significant requiring transcatheter coil occlusion. Of all variables examined, seven patients with significant decompressing collaterals had a greater transpulmonary gradient at follow-up catheterization (8 +/- 2 vs 5 +/- 2 mmHg, p=.01) and lower systemic saturations at routine clinical follow-up visits (82 +/- 5 vs 89 +/- 5 mmHg, p =.007) in comparison to the 33 others. When not evident preoperatively, decompressing venous collateral channels develop in a significant number of patients following conversion to Fontan physiology. If sufficiently large, they may produce lower than expected systemic saturations for the observed cardiac physiology. The larger decompressing channels are more likely to occur when a greater transpulmonary gradient exists postoperatively, which may require cardiac catheterization and transcatheter coil occlusion.

Isolation of the left innominate artery arising from the ductus arteriosus is a rare clinical entity with only nine cases being reported in the literature [5, 7, 8]. We describe a case consisting of tetralogy of Fallot, right aortic arch, and isolation of the left innominate artery arising from a left-sided ductus arteriosus which, to the best of our knowledge, has not been previously reported. The embryology of the aortic arch anomaly is reviewed.

We describe the first two successful cases of transcarotid balloon aortic valvuloplasty for critical aortic valve stenosis in the neonate utilizing continuous transesophageal echocardiographic guidance performed at the bedside. This method obviates the need for transporting a sick and hemodynamically unstable neonate to the catheterization laboratory and additionally reduces costs without compromising clinical care.

Objectives: The purpose of this study was to evaluate the short and intermediate term results of infants who have undergone balloon aortic valvotomy from the carotid arterial approach, and to identify risk factors in those infants who had a poor outcome., Methods: Between 1988 and 1999, balloon aortic valvotomy was attempted at four centres in 95 infants with severe aortic stenosis. Echocardiographic and hemodynamic data, and outcome, were analysed retrospectively., Results: Valvotomy was accomplished in 92 of the 95 infants, with a median age of 5 days, a range from 0 to 191 days, and weighing 3.4 kg, with a range from 1.0 to 6.5 kg. Major procedural complications occurred in 10 infants. Post-procedural aortic regurgitation was severe in 5 patients. There were 13 early deaths, and 4 late deaths. The period of mean follow-up has been 2.1 years, with a range from 0 to 9.3 years. The actuarial survival at 3 years was 76 +/- 6%. Further interventions were needed in 19 patients, giving a 3-year freedom from reintervention of 67 +/- 6%. The 51 infants who were duct-dependent were further analyzed, and found to have a higher mortality (38%) compared to those infants not dependent on the arterial duct (5%). Risk factors for a poor outcome in the duct-dependent infants were mitral stenosis (p<0.005), a left ventricle which did not form the cardiac apex (p<0.005), and an aortic valve with a diameter of less than 6 mm (p<0.05)., Conclusions: This multi-centric registry shows good results in the intermediate term for treating infants with severe aortic valvar stenosis with balloon valvotomy through a carotid arterial cutdown. Infants dependent on prostaglandin had a worse outcome, especially if they had any of the identified risk factors.

This study describes the initial hemodynamic results and early to late clinical follow-up, including magnetic resonance imaging and exercise testing, following balloon angioplasty for native coarctation of the aorta. We advocate this approach as an alternative to surgical intervention in select patients based on age, aortic arch anatomy, or in those patients who have coexisting cardiac defects that are amenable to transcatheter intervention.

A new technique is offered for selective imaging of the coronary arterial system from a transvenous approach in patients with tetralogy of Fallot as an alternative to other traditional approaches. This technique should identify important coronary anomalies while avoiding femoral arterial cannulation and compromise.

Sixty consecutive patients underwent successful transcatheter closure of patent ductus arteriosus without morbidity using a modified transvenous "snare-assisted" approach and a single elongated Gianturco coil. This technique ensures complete occlusion and avoids inadvertent embolization of the coil to undesirable sites, making transcatheter occlusion a viable alternative to surgical ligation.

Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient > 100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E1. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 +/- 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.

Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.

Objectives: This study sought to establish and maintain patency of the ductus arteriosus by a new method of transvascular formalin infiltration of the ductus arteriosus wall., Background: Maintaining patency of the ductus arteriosus postnatally is necessary with many forms of congenital heart disease to ensure survival until definitive surgical repair. A variety of approaches have to date met with variable success., Methods: Seven newborn lambs underwent catheterization at 1 to 5 days of age. A functionally closed ductus arteriosus was traversed with a specially designed porous balloon catheter, and the wall was infiltrated with 10% formalin. The ductus was then further dilated with either a 7- or 8-mm diameter balloon., Results: The ductus arteriosus wall was successfully infiltrated and dilated in all animals. Two lambs were euthanized within 24 h with congestive heart failure from a large ductus, and one lamb was electively euthanized 5 days after transvascular infiltration. Four lambs underwent serial follow-up catheterizations, one of which required repeat balloon dilation 47 days after infiltration. At latest follow-up (mean age [+/- 1 SD] 83 +/- 34 days, range 33 to 108), the pulmonary/systemic flow ratio (2.7 +/- 1.2) was unchanged from immediately after infiltration (1.9 +/- 0.5, p > 0.1). The narrowest diameter of the ductus arteriosus (4.3 +/- 0.4 mm vs. 6.9 +/- 2.6 mm, p > 0.1) and its ratio to that of the adjacent descending aorta (0.5 +/- 0.1 vs 0.4 +/- 0.1, p > 0.1) were also unchanged at latest follow-up. No systemic toxicity was observed. At postmortem study, the ductus was patent, and histologic analysis showed variable intimal and medial destruction, endothelial regeneration, loss of elastic tissue and calcification. Adjacent pulmonary artery and aorta were normal. Based on the small sample size and the observed maximal effect size of 70%, the power of the study is at most 40% to detect significant differences., Conclusions: To our knowledge, this is the first time that transvascular formalin infiltration of the ductus arteriosus wall has been applied successfully to maintain ductal patency in the newborn lamb. This method may provide a means of palliation for neonates with certain forms of congenital heart disease.

Assessment of the hemodynamic and anatomic results following balloon angioplasty of discrete native coarctation of the aorta, with particular attention to "remodeling," has required repeat cardiac catheterization and angiography, which is invasive and has limited resolution. Eight patients with hypertension and discrete native coarctation with an otherwise normally developed aortic arch underwent angioplasty at 5.0 +/- 6.8 years of age. Angiographic cross-sectional areas of the aorta indexed to body surface area at the isthmus (I), coarctation site (C), and 1 cm distal to the coarctation site (Cd) pre- and postangioplasty were compared with MRI-indexed cross-sectional areas 18 +/- 10 months (MRI-1) and 35 +/- 11 months (MRI-2) postangioplasty. From preangioplasty to MRI-2, the isthmus was smaller (149 +/- 22 versus 127 +/- 27 mm2/m2; p < 0. 05). The coarctation site was larger postangioplasty (25 +/- 9 versus 116 +/- 40 mm2/m2; p < 0.001) with continued growth at latest follow-up (116 +/- 40 versus 164 +/- 36 mm2/m2; p < 0.01). The segment 1 cm distal to the coarctation site continued to decrease in area at latest follow-up (267 +/- 78 versus 163 +/- 38 mm2/m2; p < 0. 001). I versus C versus Cd at MRI-2 were similar, whereas postangioplasty and MRI-1 cross-sectional area measurements were significantly different. Following angioplasty of discrete native coarctation, the aorta becomes more uniform or undergoes "remodeling." Noninvasive MRI is an effective means of evaluating the anatomic result following balloon angioplasty, obviating the need for repeated invasive cardiac catheterizations.

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From April 1987 to September 1993, 60 infants underwent a Norwood operation for complex congenital heart disease including hypoplastic left heart syndrome (n = 41), ventricular septal defect and subaortic stenosis with aortic arch interruption/severe coarctation (n = 7), complex single right ventricle with subaortic stenosis (n = 8), critical aortic stenosis with endocardial fibroelastosis (n = 2), and malaligned primum atrial septal defect with coarctation (n = 2). Age at operation ranged from 1 day to 3.9 months (mean 9 days, median 3.5 days). The operative mortality (< 30 days) was 33% (20 patients). Late mortality was 17% (10 patients). Nine of the 20 (45%) operative deaths occurred during the first 2 days after the operation as a result of sudden hemodynamic instability. All four infants with premature closure of the foramen ovale had pulmonary lymphangiectasia and died of pulmonary failure. Seven operative deaths have occurred in 36 patients since 1990 (19%); in the past 2 years, no operative deaths have occurred in 22 patients. Overall, there are 30 long-term survivors (50%). Twenty-one of these 30 infants have undergone a two-stage repair with a modified Fontan operation at 7.3 to 27.6 months of age (mean 18.1 months) with no mortality. Six patients have entered a three-stage repair strategy by undergoing a hemi-Fontan procedure at 6.8 to 23.0 months (mean 8.8 months) with no mortality, and two of these patients have now had their modified Fontan operation at 23.0 to 46.7 months of age with no mortality (four are still awaiting surgery). Two patients have undergone a two-ventricle repair with a Rastelli procedure, with no mortality at 7.4 and 14.1 months of age. Early in our experience, infants undergoing the Norwood operation had a high early mortality most often related to sudden hemodynamic instability. After we instituted a protocol that adds carbon dioxide to the inspired gas during postoperative mechanical ventilation, the postoperative course became more stable and there have been no operative deaths. In summary, the operative mortality for the Norwood operation continues to improve. A subsequent Fontan operation can be performed with excellent clinical results.

Hypertrophic cardiomyopathy and abnormal ventricular diastolic filling in the infant of the diabetic mother is related to poor maternal glycemic control. Evaluation of fetuses of well controlled diabetic mothers has not been examined. Eleven fetuses of nondiabetic mothers (normals) and 9 fetuses of well controlled insulin-dependent diabetic mothers (FODMs) underwent serial evaluation of cardiac growth and ventricular diastolic filling using M-mode and Doppler echocardiography at 20-26 weeks' (period 1), 27-33 weeks' (period 2), 34-40 weeks' (period 3),m and 48-72 hours after birth (period 4). Indices of right and left ventricular diastolic filling included time velocity integral ratios (E/A and %E/E+A). Cardiac growth and birth weight in the two groups were similar consistent with "good" glycemic control. This conclusion was supported by similar maternal glycosylated hemoglobin (%A1C) prenatally and newborn %A1C and C-peptide values postnatally. Heart rate before and after birth and placental resistance prenatally were similar. Both normals and FODMs demonstrated an increase in left ventricular E/A and %E/E+A ratios from period 1 to 4 (p < 0.0001). This shift occurred earlier (by period 2) in normals (p < 0.01). Right ventricular filling ratios increased by period 4 in normals only (p < 0.01). No differences were noted between the groups during any period. Good glycemic control in FODMs results in normal cardiac growth and ventricular diastolic filling. Progression of diastolic filling is abnormally delayed, however, and is presumably more exaggerated in poorly controlled diabetics.

We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insufficiency occurred following placement of an endocardial pacing lead, and severe mitral insufficiency occurred acutely at 2.5 months of age. Both valves were noted at surgery to have fibrotic and calcific chordae tendineae that had ruptured from their papillary muscles. An inflammatory reaction within the fetal heart related to transplacental passage of Sjögren's antibody was likely responsible.

Neutropenia is an infrequent complication following administration of the angiotensin-converting enzyme (ACE) inhibitor, captopril. Most reports have been in adult patients, with rare reports in the pediatric population. We report two cases of neutropenia following captopril use in cardiac patients with trisomy 21. As this was not seen in patients without Down's syndrome, we postulate that patients with trisomy 21 have bone marrow which is "at risk" for suppression, and, thus warrant close evaluation while on such medications.

The mechanism for exercise systolic hypertension after a "good" operative repair of coarctation of the aorta remains speculative. Twenty-four children (mean age +/- SD 10.3 +/- 3.8 years) were studied with continuous-wave Doppler echocardiography while they performed continuous, graded, maximal treadmill exercise. Patients were free of "recoarctation" based on conventional resting echocardiography. Measurements of ascending and descending aortic peak instantaneous systolic velocity were obtained at rest, throughout exercise and during recovery. Results were compared with 24 age- and gender-matched control subjects. Fifteen patients were normotensive (group 1) (peak systolic blood pressure, 147 +/- 21 mm Hg) and 9 developed systolic hypertension during exercise (group 2) (196 +/- 32 mm Hg) (p < 0.05) (control subjects, 143 +/- 21 mm Hg). Descending aortic peak systolic velocity at rest ranged from 1.50 +/- 0.27 m/s in the control group to 2.57 +/- 0.57 m/s (group 1) and 2.93 +/- 0.43 m/s (group 2) (p < 0.05, group 2 vs control). Differences were amplified at peak exercise with systolic velocity increasing to 4.26 +/- 0.61 m/s in group 2 but only to 3.61 +/- 0.70 m/s in group 1 and 2.26 +/- 0.38 m/s in control subjects (p < 0.05, group 2 vs group 1 and control). Seven patients developed a descending aortic diastolic velocity during exercise. Stepwise linear regression analysis identified 2 variables to be significant determinants of peak exercise systolic blood pressure in the "total" patient group: (1) age at exercise testing, and (2) descending aortic peak systolic velocity at peak exercise (r2 = 0.88, p < 0.001) (group 2, alone - r2 = 0.98, p < 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Objectives: This study was conducted to evaluate the incidence and etiology of hypertension and aortic arch gradients during exercise in patients who have apparent good coarctation repair assessed at rest., Background: The reported incidence of recurrent aortic arch obstruction (rest gradient > 20 mm Hg) after previous successful surgical repair varies from 0% to 60% and usually is associated with recurrent stenosis at the site of surgical repair., Methods: Maximal treadmill exercise with Doppler echocardiographic gradient estimation was performed in 28 patients with a good coarctation repair at rest (normal blood pressure and arch gradient < 20 mm Hg) who had isolated coarctation repair a mean of 7.8 years previously., Results: Eight (29%) developed systolic hypertension for age and a mean Doppler gradient of 45 +/- 13 mm Hg. At cardiac catheterization, the rest peak to peak systolic gradient (6 +/- 6 to 28 +/- 7 mm Hg, p < 0.001), peak systolic instantaneous gradient (16 +/- 11 to 48 +/- 9 mm Hg, p < 0.01) and cardiac index (3.5 +/- 0.7 to 5.9 +/- 1.1 liters/m per m2, p < 0.001) all increased during isoproterenol infusion. Angiographic systolic aortic arch measurements proximal to the innominate artery, left common carotid artery, left subclavian artery and the narrowest dimension at the coarctation repair site demonstrated hypoplasia at the left common carotid artery (11.8 +/- 1.7 vs. 16.7 +/- 2.9 mm/m2, p < 0.01) and left subclavian artery (11.6 +/- 1.7 vs. 15.4 +/- 3.1 mm/m2, p < 0.05) compared with findings in 10 patients with normal aortograms. Transverse aortic arch ratios were also smaller in the eight patients with abnormal findings. Preoperative angiographic ratios were not predictive of late postoperative findings., Conclusions: Exercise testing detects hypertension and arch gradients in patients with a good coarctation repair as assessed at rest. The hypertension and arch "obstruction" appear to be related to discrepancies in the growth of the transverse aortic arch proximal to the repair site, rather than a "recoarctation" of the aorta.

Our experience with congenital supravalvular aortic stenosis dates from 1977 to 1991. Thirteen patients, aged 2 days to 38 years (mean 7.6 years, median 3.8 years), had surgical repair of the lesion. The preoperative peak-to-peak systolic gradients ranged from 25 to 110 mm Hg (mean 64 mm Hg). Four patients had trace to mild aortic insufficiency. Surgical repair was accomplished by several techniques that have evolved over time. There was one death in a 2-day-old neonate who also had severe hypertrophic cardiomyopathy. The 12 survivors had postoperative gradients of 0 to 30 mm Hg (mean 10 mm Hg) in the supravalvular region. The mean reduction in gradient was 48 mm Hg. A new technique employing all autologous aortic tissue is described.

Preoperative balloon atrial septostomy is the standard therapy for babies with uncomplicated cyanotic dextrotransposition of the great arteries despite the effectiveness of prostaglandin E1 infusion in alleviating systemic hypoxemia and the reported success of arterial switch repair during the 1st weeks after birth. The clinical records and echocardiographic findings of 23 infants (mean birth weight +/- SD 3.3 +/- 0.5 kg) with uncomplicated transposition of the great arteries were analyzed. Fifteen infants (Group I) did not undergo septostomy, and 8 (Group II) underwent septostomy. Before prostaglandin infusion, mean arterial oxygen tension (Po2) in Group I (26 mm Hg) did not differ from that in Group II. After prostaglandin infusion, Po2 increased significantly in Group I (43 +/- 8 mm Hg, p less than 0.001) but not in Group II despite a widely patent ductus and predominant left to right ductal shunt in all. After septostomy, Po2 increased significantly (43 +/- 4 mm Hg, p less than 0.03), and did not differ from that in Group I. Echocardiographic features generally demonstrated a nonrestrictive foramen ovale in Group I and a restrictive foramen ovale in Group II. The latter was associated with persistent hypoxemia after prostaglandin. Thus, the diameter of the foramen ovale was the primary factor influencing arterial oxygenation during prostaglandin infusion. Babies underwent the arterial switch operation at a mean age of 70 +/- 65 h with an overall survival rate of 96%; there was only one postoperative death (Group II). Absence of septostomy had no negative influence on any postoperative variable, including duration of ventilatory and inotropic support, time to discharge, or mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

Young age remains a reported risk factor for a successful Fontan operation despite improved survival rates. Since March 1978, the Fontan operation has been performed in 47 patients. To avoid a primary or secondary palliative shunt, an early Fontan procedure (Group 1: mean age 1.5 +/- 0.5 years, range 0.6 to 2) has been performed in 17 children with the outcome similar to that of the remaining 30 older patients (Group 2: mean age 7.5 +/- 5 years, range 2.4 to 23 years). Preoperatively both groups had acceptable hemodynamic status for a successful Fontan result. Operative variables including cardiopulmonary bypass time, aortic cross-clamp time and core temperature were similar between groups and did not affect mortality. The postoperative mortality rate including early surgical (0% vs. 13%, respectively), late (18% vs. 12%) and total (18% vs. 23%) was similar between Groups 1 and 2 (p greater than 0.05). Immediate postoperative arrhythmias were more frequent in Group 1 (71% vs. 25%, p less than 0.01) with no related mortality, while late arrhythmias occurred with equal frequency (29% vs. 39%, p greater than 0.05). Group 1 infants required a longer hospital stay (22 +/- 9 vs. 14 +/- 5 days, p less than 0.01). Thus, young age is not a risk factor for successful outcome of the Fontan operation in patients with acceptable preoperative hemodynamic status. An early Fontan operation may also avoid prolonged palliative procedures and their potential deleterious effects.

Intraoperative echocardiography is gaining increasing acceptance in the assessment of the surgical repair of congenital heart defects. Because of its ideal imaging location, intraoperative transesophageal echocardiography (TEE) has been especially helpful in evaluating pulmonary venous return and the integrity of the left atrioventricular valve following mitral valvuloplasty, complete atrioventricular valve repair and correction of complex congenital heart disease. It has not been routinely used in infants and small children because of a prohibitively large probe size. We report the successful use of a recently developed "pediatric" TEE probe capable of Doppler and color flow imaging in two patients less than 7 years of age. No complications were encountered. Recommendations regarding optimal probe imaging positions in infants are provided.

To evaluate cardiac growth in fetuses of those diabetic mothers with good metabolic control, we examined M-mode echocardiographic measurements obtained from 24 fetuses of diabetic mothers (FODM) and compared these with measurements from 31 normal fetuses of similar gestational age. Fetuses were grouped into three gestational periods: 20 to 26 weeks, 27 to 33 weeks, and 34 to 40 weeks. The mothers were believed to have good metabolic control on the basis of mean daily glucose profiles and glycosylated hemoglobin A (HbA1C) values of approximately 110 mg/dl (610 mumol/L) and 7.5%, respectively, before fetal scanning, and estimated fetal weight similar to that of normal fetuses during all three gestational periods. Both FODM and normal fetuses had significant increases in M-mode measurements from period 1 to period 3, but in FODM, cardiac hypertrophy developed by late gestation (period 3). This involved the interventricular septum (6.1 +/- 0.7 vs 4.9 +/- 0.3 mm, p less than 0.05), right ventricular free wall (5.7 +/- 0.8 vs 3.2 +/- 0.3 mm, p less than 0.01), and left ventricular free wall (6.4 +/- 0.6 vs 3.3 +/- 0.4 mm p less than 0.01). The interventricular septum/right ventricular free wall ratio was similar, whereas the interventricular septum/left ventricular free wall ratio in FODM was smaller by period 3 (1.0 +/- 0.1 vs 1.6 +/- 0.1, p less than 0.05). The right ventricular diastolic dimension was similar, but the left ventricular diastolic dimension was significantly smaller in FODM during periods 2 and 3 (8.2 +/- 1.2 vs 12.2 +/- 0.7 mm, p less than 0.05). Strict metabolic control did not prevent FODM from having abnormal cardiac growth. We conclude that good metabolic control results in normal estimated fetal weight but that FODM remain at risk for mild global cardiac hypertrophy and altered diastolic dimensions.

We reviewed our experience in 38 patients who underwent a Fontan operation. In the first five patients ages 7.5 to 23 years (mean, 15 years), a conduit was placed from the right atrium to the small right ventricle or the pulmonary artery (PA). The remaining 33 patients, ages 7 months to 14 years (mean, 4.8 years), had a modified Fontan operation with direct systemic venous or right atrial to PA anastomosis. The diagnoses were tricuspid atresia (n = 14), single ventricle (n = 10), hypoplastic right or left ventricle (n = 9), double-outlet right ventricle with inlet ventricular septal defect and pulmonary atresia or stenosis (n = 3), criss-cross ventricles and transposition of the great arteries (n = 1), and atrioventricular canal and anomalous pulmonary venous connection (n = 1). Thirty-two patients had previous surgery. Other procedures included PA banding (n = 7), systemic to PA shunts (n = 25), Norwood operation (n = 3), and a Damus-Kaye-Stansel anastomosis (n = 1), repair of total anomolous pulmonary venous connection (n = 1), a Blalock-Hanlon atrial septectomy (n = 1), and enlargement of a restrictive ventricular septal defect (n = 1). There were four operative deaths (10.5%), three from low cardiac output and one from subaortic obstruction. There were no deaths in patients younger than 3 years of age (n = 13). Subaortic obstruction developed in six of the seven patients who had pulmonary artery banding and resulted in three deaths. In our experience, diagnosis, previous surgery, type of previous operation, PA pressure, and younger age are not risk factors for early or late death. Subaortic obstruction is a major risk factor for late death. Accordingly we now perform a Damus-Kaye-Stansel anastomosis combined with a systemic to PA shunt in those children with excessive pulmonary blood flow who anatomically are likely to develop subaortic obstruction. A modified Fontan operation can be performed any time after 1 year of age and in some patients after 6 months of age, providing the anatomy and physiology of the patient are acceptable.