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Due to the presence of fetal haemoglobin and the patency of anatomical shunts the fetal circulation is perfectly adapted to intrauterine life, utilizing the placenta as the organ of gas exchange. Immediate adaptation must occur at birth as the lungs take over this role. Initially the pulmonary vascular resistance is high but pulmonary blood flow progressively increases in the newborn due to vasodilatation as a consequence of increased oxygenation and activation of pulmonary stretch receptors. Changes in the pulmonary vasculature are mediated through a number of vasoactive substances, including nitric oxide. Increased pulmonary blood flow leads to an increase in left atrial pressure with subsequent closure of the foramen ovale. In some babies this normal adaptation fails and persistent fetal circulation may develop, leading to the symptoms of persistent pulmonary hypertension of the newborn. The mainstay of treatment is to maximize oxygenation by use of an appropriate ventilatory strategy and to maintain blood pH within normal range using inotropic support as indicated to sustain an adequate systemic blood pressure. Nitric oxide has proved to be an extremely effective treatment for this condition in some cases but ECMO may be required as a rescue therapy. Persistent pulmonary hypertension of the newborn still carries a 10–20% mortality rate despite modern intervention.

Patent foramen ovale (PFO) is associated with cryptogenic stroke, migraine headache, decompression sickness, and platypnea-orthodeoxia syndrome. Patients undergoing transesophageal echocardiography are often hypovolemic from preprocedural fasting and might not demonstrate right to left shunting owing to insufficient right atrial pressure generation, despite provocative maneuvers. We hypothesized that volume replenishment with saline loading could potentially unmask a PFO by favorably modulating the interatrial pressure gradient. Our study sought to examine the role of pre- or intraprocedural intravenous fluid replenishment on PFO detection during transesophageal echocardiography. A total of 103 patients were enrolled. An initial series of bubble injections was performed unprovoked and then with provocative maneuvers such as the Valsalva maneuver and coughing. The patients were then given a rapid 500 ml saline bolus, and the same sequence of bubble injections was repeated. The presence, type, and magnitude of the right to left shunts were noted before and after the saline bolus. The detection rate of PFO increased from 10.6% to 26.2% after saline loading without any provocative maneuvers. When combined with provocative maneuvers (Valsalva or cough), saline loading improved the detection rate from 17.4% to 32.0%. Overall, from amongst the 103 enrolled patients, saline bolusing resulted in a de novo diagnosis of PFO in 15 patients, atrial septal aneurysm in 15, PFO coexisting with an atrial septal aneurysm in 10, and pulmonary arteriovenous fistula in 5 patients. In conclusion, saline infusion in appropriately selected patients during transesophageal echocardiography significantly enhances the detection of PFOs and pulmonary arteriovenous fistulas.

The association between the anatomic characteristics obtained by omni-plane transesophageal echocardiography (TEE) and stroke recurrence in patients with cryptogenic stroke and patent foramen ovale (PFO) remains unclear. In the present longitudinal follow-up study, we sought to investigate whether PFO findings assessed by TEE can predict stroke recurrence. Of the 1,014 consecutive patients with acute ischemic stroke referred for TEE, 184 (mean +/- SD age, 51 +/- 14 years) were classified as having cryptogenic stroke with PFO, and follow-up data were available for 181 patients. During follow-up (median 3.5 years), 14 patients (7.7%) experienced stroke recurrence. Multivariate analysis showed that atrial septal aneurysm or hypermobility of the atrial septum (hazard ratio 6.04, 95% confidence interval 1.84 to 19.86, p = 0.003) and PFO size (hazard ratio 3.00, 95% confidence interval 1.96 to 4.60, p

We prospectively compared the bubble test with agitated saline for right-to-left shunt using transcranial Doppler (TCD) of the right middle cerebral artery and second harmonic imaging duplex of the right common carotid artery (CCA) in 100 consecutive patients. Microembolic signals (MES) were counted offline. MES were classified into 6 classes: absent (class 0), 1-10 MES (class 1), 11-20 MES (class 2), 21-30 MES (class 3), 31-50 MES (class 4) and >50 MES or "curtain effect" (class 5). For TCD, classes 2-5 combined (i.e., "large" shunts), the sensitivity of duplex with the Valsalva maneuver was 95.3%, the specificity was 100%, the positive predictive 100%, the negative predictive value 96.6% and accuracy 98.0%. Second harmonic imaging duplex of the CCA may substitute TCD for the bubble test when an adequate cranial bone window is not available. This technique may also greatly increase the number of facilities where the bubble test can be carried out. However, tests with few or no MES need to be confirmed by TCD or transesophageal echocardiography.

Transcatheter patent foramen ovale (PFO) closure might be effective in improving migraines. To assess the efficacy of PFO closure in migraineurs with a moderate to large right-to-left shunt and instrumental evidence of embolic cerebral damage, 76 highly symptomatic migraineurs were prospectively investigated. The presenting clinical syndrome was stroke in 16 patients, repeated transient ischemic attack in 32 patients, and lone migraine associated with cerebral ischemic lesions on magnetic resonance imaging in 28 patients. Migraine severity was assessed before PFO closure and monthly for 6 months after discontinuation of antiplatelet therapy. At the end of 12 months of follow-up, the averaged postprocedural total score was compared with the baseline score. Transcatheter PFO closure was successful in all patients, and the 12-month PFO closure rate was 97%. The baseline total migraine score was similar in patients with stroke, transient ischemic attack, and lone migraine (6.8 +/- 1.6, 6.7 +/- 1.4, and 6.9 +/- 1.7 respectively, p = NS). After a mean follow-up of 13.7 +/- 2.4 months, no recurrent cerebrovascular episodes had occurred. At the end of the follow-up period, a significant reduction in the total migraine score was observed in all groups, regardless of the initial clinical presentation. Migraine was completely abolished in 35 patients (46%), improved in 27 (36%), and unchanged in 14 (18%). The proportion of patients with migraine suppression and improvement was similar in the 3 groups. In conclusion, in highly symptomatic migraineurs with previous ischemic cerebral events and instrumental evidence of cerebral embolism, transcatheter PFO closure can result in improvement of migraine severity in a high percentage of patients. (C) 2009 Published by Elsevier Inc. (Am J Cardiol 2009;104:434-439)

Congenital diaphragmatic hernia (CDH) is characterized by a defect in the diaphragm that permits abdominal viscera to herniate into the chest. These herniated viscera are thought to compress the growing lung and cause lung parenchymal and vascular hypoplasia. The genetic defects that cause the diaphragmatic defect may also contribute primarily to lung hypoplasia. Postnatal reduction of the herniated abdominal viscera and correction of the diaphragmatic defect are easily achievable, but the lung hypoplasia persists, often leading to persistent fetal circulation and respiratory failure. This article reviews the experimental basis of fetal therapy for CDH and the US clinical experience with tracheal occlusion.

Patent foramen ovale (PFO) is a remnant of the foetal circulation, found in about a quarter of the population. PFO is an asymptomatic condition and the high prevalence infers that it is in most cases of no or only limited clinical significance. However, recent research has found an increased prevalence of PFO in cryptogenic stroke, decompression illness and migraine. The presence of a PFO has also been associated with oxygen desaturation in conditions such as obstructive pulmonary disease and obstructive sleep apnoea. The rapid evolution and widespread availability of catheter-based closing techniques have further stimulated interest. The seemingly growing significance of PFO will be discussed in this review.

Meconium aspiration syndrome (MAS) mainly affects term and post-term infants. It ranges from mild respiratory distress to life-threatening cardiorespiratory failure. Prevention and treatment strategies are evolving. Amnioinfusion is no longer recommended for women with meconium staining of the amniotic fluid (MSAF). Pharyngeal suction prior to delivery of the fetal shoulders and tracheal suction of vigorous infants after birth do not prevent MAS and may be harmful. Tracheal suction is recommended for non-vigorous infants with MSAF but has not been studied prospectively. Bolus surfactant therapy, high frequency oscillatory ventilation and inhaled nitric oxide may be beneficial in severe cases. Surfactant lavage reduces the combined risk of death or requirement for extracorporeal membrane oxygenation (ECMO) when compared with no surfactant treatment but its role in the routine management of MAS is uncertain and it should be compared with bolus surfactant in prospective studies. ECMO reduces the risk of mortality in severe cases so it is of paramount importance to discuss the transfer of severely ill infants to an ECMO centre.

Summary Background Patent foramen ovale is an anomaly responsible for paradoxical embolizations and cerebral ischemic events. Aims We want to show second harmonic transthoracic echography sensitized by contrast agent perfusion is as well as transesophageal echography to patent foramen ovale detection. Methods Onene hundred twenty one patients referred for transesophageal echocardiography for patent foramen ovale detection, underwent additive second harmonic transthoracic echocardiography with one of three randomized contrast agents: a mixture A of dextrose and air, mixture B of dextrose and air and blood, or mixture C of hydroxyethylamidon. The severity of atrial shunting was evaluated on recordings by semi-quantitative scoring. Intensity of contrast was also assessed by objective quantitative videodensitometry. Results No difference was observed between the two techniques, nor between mixture A, B and C in terms of PFO detection during each exam. However, quantitative contrast analysis showed higher intensity with mixtures B and C with mixture A during transthoracic echography. Conclusions When performed with a contrast agent, second harmonic transthoracic echography and transoesophageal echography are comparable when it comes to patent foramen ovale detection. Although the composition of the contrast agent does not appear to affect the rate of this detection, contrast quality in the right atrium during transthoracic exam is better with mixtures B and C than with mixture A.

The aim of this study was to assess (1) the incidence of patent foramen ovale (PFO) in carcinoid syndrome (CS) and (2) the feasibility of percutaneous closure procedure in selected patients with CS. One hundred eight patients were prospectively studied: 54 with CS and an age- and gender-matched control group. All patients underwent conventional and contrast echocardiography. Patients with clinical signs of dyspnea (New York Heart Association classor =III), cyanosis, carcinoid heart disease (CHD), and severe PFO were referred for the percutaneous closure of PFO. The prevalence of PFO was 41% in patients with CS and 22% in the control group (p = 0.03) and was significantly higher in patients with CHD (59%, p = 0.009). Four patients (14% of those with CHD) were referred for the percutaneous closure of PFO, and 3 patients ultimately underwent PFO closure (using Amplatzer septal occluders). At 6-month follow-up, New York Heart Association class was improved in all patients, as well as arterial blood gas results (p = 0.04) and 6-minute walking distance (p = 0.03), but all patients presented residual right-to-left shunts. In conclusion, this prospective study demonstrates that in patients with CHD, the prevalence of PFO is high and that percutaneous closure of PFO is feasible, with a reduction in symptoms but with residual shunting.

The management of a patient with hip fracture during general anesthesia, who developed severe intractable hypoxemia caused by intraoperative pulmonary embolism in the presence of undiagnosed patent foramen ovale, is described. The role of urgent intraoperative transesophageal echocardiography in situations where acute perioperative pulmonary embolism/patent foramen ovale is suspected is emphasized.

Recent epidemiological data suggest a bidirectional link between patent foramen ovale (PFO) and migraine with aura (MA) with a relative risk of 2 for PFO in subjects with MA and for MA in subjects with PFO. There is no evidence for a link between PFO and migraine without aura. This link is not systematic and applies only to subsets of PFO, mostly large ones, and to subsets of patients with MA. Although comorbidity cannot be ruled out, it may be that this link is partly causal and that some large PFOs may favor MA attacks in genetically predisposed subjects, by allowing vasoactive substances, platelet emboli or paradoxical emboli to bypass the lung filter and trigger the cortical spreading depression of the aura. The first double blind randomised trial of PFO closure in refractory MA, "MIST", has failed to show a benefit on the primary efficacy end point: cessation of attacks during the analysis period included between 3 and 6 months after the procedure. There is thus at present no scientific reason to look for PFO or to close PFO in migraine patients.

The prevalence of a right-to-left shunt, both cardiac and pulmonary, is high in patients with migraines, especially migraine with aura. Percutaneous closure of a right-to-left shunt seems to be associated with a pronounced decrease in the number of migraine attacks or its prevalence. In this review, the relationship between migraine and right-to-left shunting is described by highlighting the different pathophysiologic hypotheses.

OBJECTIVES We studied the value of cardiac magnetic resonance imaging (CMRI) before and after closure of patent foramen ovale (PFO) in patients with cryptogenic ischemic events. BACKGROUND Cardiac magnetic resonance imaging is a powerful noninvasive tool for detailed assessment of cardiac anatomy and function. The relevance of CMRI compared with transesophageal echocardiography (TEE) in patients undergoing transcatheter PFO closure has not been evaluated so far. METHODS Contrast-enhanced CMRI and TEE were performed in 75 patients before and after PFO closure. Twelve months after PFO closure, both imaging techniques were repeated in 61 patients with contrast application. To determine provokable atrial right-to-left shunting in CMRI, we applied a contrast-enhanced perfusion imaging technique. Detection of atrial septal aneurysm (ASA) was achieved by means of a high-resolution cine imaging technique. RESULTS Before PFO closure, ASA was seen with CMRI in 28 of 75 cases (37.3%), compared with 47 of 75 (62.7%) cases using TEE. There were a total of 211 CMRI studies with a corresponding TEE performed in 75 patients. No shunt was present in 107 of 211 studies with both techniques. Contrast-enhanced right-to-left shunting was detected by CMRI in 48 of 72 (66.6%) cases with moderate or severe shunts seen with TEE, but only in 6 of 32 (18.8%) studies with mild shunts with TEE. Anomalous venous returns were excluded in all patients. In two patients, coronary anomalies were seen. CONCLUSIONS The present CMRI technique is inferior to TEE in detection of contrast-enhanced right-to-left shunting and identification of ASA.

OBJECTIVE To describe a multicenter experience with patent foramen ovale (PFO) and atrial septal defect (ASD) device closure for presumed paradoxical emboli in children and young adults ( PATIENTS AND METHODS Medical records were reviewed of patients who had device closure of an ASD or PFO, who were younger than 35 years, and who had a history of presumed paradoxical embolus between January 1999 and August 2005 at Mayo Clinic, Rochester, Minn, University of Parma, Parma, Italy, and Loyola University Medical Center, Maywood, Ill. RESULTS Forty-five patients fulfilled the inclusion criteria. Median patient age was 29.0 years (range, 5.0-34.9 years), and 23 patients (51%) were male. Clinical diagnoses included the following: stroke, 30 (67%); transient ischemic attack, 13 (29%); myocardial infarction, 1 (2%); and renal infarct, 1 (2%). Overall, 42 patients (93%) had a PFO, and 3 (7%) had an ASD. Seventeen patients had known cardiovascular disease risk factors: tobacco use (10 patients), hypercoagulable states (7 patients), systemic hypertension (3 patients), and hyperlipidemia (2 patients). No major procedural complications occurred. Median follow-up evaluation was performed at 5.3 months (range, 2.5-40.0 months). Forty-four patients (98%) had no recurrent neurologic events and no residual atrial shunt by contrast transthoracic echocardiography. CONCLUSIONS Cryptogenic ischemic events occur in young patients and have serious sequelae. The potential for paradoxical embolization through a PFO or an ASD should be assessed in all such patients. In our short-term follow-up, device closure was a safe alternative therapeutic option for children and young adults with presumed paradoxical emboli.

Platypnea-orthodeoxia syndrome is a rare cause of hypoxaemia per or postsurgery. This one is characterized by right-to-left intracardiac shunting responsible of postural hypoxemia in orthostatic position, which is improved by supine position. We described the first case of a platypnea-orthodeoxia syndrome occurred during laparoscopic surgery.

Purpose The prognosis of antenatally diagnosed congenital diaphragmatic hernia (ADCDH) is still very poor despite of innovation of various therapeutics. The authors reviewed their new therapeutic strategy of ADCDH from a viewpoint of cardiologic function. Methods The cardiac function in 19 cases of ADCDH was reviewed. The patients, at the age of 0 days, were divided into 2 groups, PG (+) and PG (−), according to the requirement of prostaglandin E 1 (PGE 1 ) to attenuate pulmonary hypertension. The left ventricular (LV) end-diastolic dimension (LV diastolic diameter index [LVDI]) and bilateral pulmonary arterial diameters (total pulmonary artery index [TPAI]) were measured on days 0 and 2. Result Only 1 patient died of cardiac or respiratory failure, and the survivors' postoperative course was uneventful. Eleven patients needed inhalation of nitric oxide (NO), and in 9 of those, PGE 1 was administered. The LVDI and TPAI of day 0 in PG (+) were significantly smaller than those in PG (−) and the controls. The LVDI increased from postnatal day 0 to day 2 in both PG (+) and PG (−). Although the LV was too small to output enough volume, the right ventricle successfully compensated for the low output through the ductus arteriosus, kept patent by NO and PGE 1 . Conclusion For ADCDH with sever pulmonary hypertension, keeping patent ductus arteriosus with NO and PGE 1 plays a critical role in obtaining excellent clinical outcome. Thus, the authors proposed a new therapeutic strategy for ADCDH based on a circulatory management.

Patients with patent foramen ovale and cryptogenic stroke are at risk of recurrence, but there are few data on strategies to prevent this.We studied 280 consecutive patients with cryptogenic cerebrovascular events and a patent foramen ovale examined by multiplane transesophageal echocardiography. Patients received platelet inhibitors (n = 66) or anticoagulation (n = 47) or underwent device closure (n = 167). During the mean follow-up of 2.6 years, we compared the frequency of recurrent events, death and severe treatment complications.A total of 33 (12%) patients had a recurrent cerebrovascular event. The annual recurrence rates were 13% in patients treated with platelet inhibitors, 5.6% in those on oral anticoagulation, and 0.6% in those after device closure. Independent predictors of recurrent cerebrovascular events were a patent foramen ovale larger than 4 mm (hazard ratio 3.8, 95% CI 1.2-11.2; p = 0.017) or previous strokes (hazard ratio 4.3, 95% CI 2.0-9.2; p0.001). Interventional closure of the patent foramen ovale decreased the risk of a recurrent event compared with oral anticoagulation (hazard ratio 0.06, 95% CI 0.12-0.29; p0.001), whereas antiplatelet therapy showed a trend to an increased risk (hazard ratio 2.3; 95% CI 0.9-5.5; p = 0.055). Major side effects occurred in 7 anticoagulated patients and in 13 patients after device closure. There were two non-vascular deaths and one fatal hemorrhagic stroke.Patients with a large patent foramen ovale and a cryptogenic cerebrovascular event had a substantial risk of recurrence even with medical treatment. Risk of recurrence was lower after device closure of the patent foramen ovale.

The incidence of foramen ovale restriction or closure is greater in fetuses with congenital heart defects (CHDs) and obligate atrial shunting and/or left atrial hypertension. Foramen ovale restriction or closure in fetuses with CHD can cause distress in utero or at birth. We suggest performing serial ultrasound assessment for at-risk fetuses with CHD to best assure fetal well-being plan the timing and mode of delivery, and develop management protocols for in utero and postnatal care. The future for these patients may be more optimistic as fetal intervention procedures are developed and mastered.

ObjectivesWe sought to determine whether direct foramen ovale (FO) assessment or pulmonary venous (PV) flow patterns in fetal hypoplastic left heart syndrome (HLHS) correlate with clinical markers of postnatal left atrial (LA) hypertension severity associated with restrictive FO.BackgroundRestrictive FO places a newborn with HLHS at high risk of mortality and morbidity.MethodsWe reviewed the prenatal and postnatal echocardiograms and outcomes of 45 fetuses with variants of HLHS diagnosed since May 1999 to determine whether direct FO assessment or PV flow patterns correlate with clinical LA hypertension after birth.ResultsDirect FO assessment in utero showed a poor correlation with postnatal FO size, Pao2, base excess, and the need for atrial septoplasty (p > 0.05). In 40 fetuses with available PV spectra, three PV flow patterns were observed: 1) continuous forward flow with a small a-wave reversal (velocity time integral [VTI] for reverse/forward flow [VTIR/VTIF ratio

Objective To review our experience with, and profile the safety and efficacy of, the Amplatzer PFO (patent foramen ovale) occluder (APO) and Amplatzer septal occluder (ASO) used to close PFO and/or atrial septal defect (ASD) in patients with paradoxical embolism (PE). Patients and Methods Between April 1998 and November 2002, 103 patients at the Mayo Clinic in Rochester, Minn, and Scottsdale, Ariz, mean age 52.4 years, with presumed PE (transient ischemic attack [n=22], stroke [n=77], or peripheral emboli [n=4]) underwent transcatheter device closure of PFO (n=81), ASD (n=12), and ASD/PFO (n=10) with 106 devices (APO [n=22] or ASO [n=84]). Results All devices deployed successfully, and no patients died. Procedural complications included atrial fibrillation (n=2), vessel injury (n=3), profound sinus node dysfunction (n=1), and device embolization with successful retrieval (n=1). At 3 months, 7 of 95 monitored patients had trivial residual shunt; at 12 months, 2 of 28 monitored patients had trivial residual shunt. Three patients had recurrent events—2 transient ischemic attacks and 1 retinal artery occlusion—at a mean ± SD follow-up of 8.3±8.1 months (range, 1-34 months). None of these 3 patients had residual shunt or evidence of intracardiac thrombus. The average annual recurrence of all events was 3.6% at 23 months. The overall mean ± SD freedom from recurrence of all events was 98.9%±1.2% and 83.8%±10.2% at 12 and 29 months of follow-up, respectively. Conclusions Transcatheter device closure of PFO and/or ASD with use of APO/ASO in patients with presumed PE is effective and safe. Recurrent events may occur in the absence of a residual shunt.

Objectives We assessed the use of intracardiac echocardiography (ICE) as the primary means for both selection of the Amplatzer Septal Occluder (ASO) and the guidance of transcatheter closure of secundum atrial septal defects (ASDs). Background The standard method for transcatheter closure of ASDs requires balloon-sizing maneuver and transesophageal echocardiographic (TEE) monitoring. The role of ICE during transcatheter closure of ASDs has not yet been established. Methods In 91 patients with ASDs, two standardized orthogonal sections were used to obtain ICE-derived measurements of the fossa ovalis and to assess optimal device deployment: the transverse section on the aortic valve plane, and the longitudinal section on the four-chamber plane. Results In all patients, ICE planes were identified with excellent resolution, providing proper measurements of the fossa ovalis, from which to derive geometric assumptions for the selection of an appropriately sized device. The ASO waist diameter was chosen on the basis of the r value ( r = c 2 + p 2 , where r is the radius of an ideal circle that intersects the elliptical fossa ovalis in its semi-latus rectum, c is the foci half-distance of the fossa ovalis, and p is its semi-latus rectum). During the procedure, the four-chamber plane allowed us to obtain easily interpretable images of all stages of device deployment. Midterm complete occlusion rate was 97.8%. No ICE-related complications occurred. Conclusions The ICE evaluation of ASDs allows quantitative and qualitative information for both proper ASO selection and optimal device placement, thus eliminating the cumbersome balloon-sizing maneuver and the need for general anesthesia during TEE monitoring.

Objectives This study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children. Background Results of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children. Methods Between December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age ≤5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter). Results The mean age at closure was 3.6 ± 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 ± 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly. Conclusions In the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children.

Objectives This study sought to compare the safety, efficacy and clinical utility of the Amplatzer septal occluder (ASO) for closure of secundum atrial septal defect (ASD) with surgical closure. Background The clinical utility of a device such as the ASO can only be judged against the results of contemporaneous surgery. Methods A multicenter, nonrandomized concurrent study was performed in 29 pediatric cardiology centers from March 1998 to March 2000. The patients were assigned to either the device or surgical closure group according to the patients’ option. Baseline physical exams and echocardiography were performed preprocedure and at follow-up (6 and 12 months for device group, 12 months for surgical group). Results A total of 442 patients were in the group undergoing device closure, whereas 154 patients were in the surgical group. The median age was 9.8 years for the device group and 4.1 years for the surgical group (p 0.05). The complication rate was 7.2% for the device group and 24.0% for the surgical group (p Conclusions The early, primary and secondary efficacy success rates for surgical versus. device closure of ASD were not statistically different; however, the complication rate was lower and the length of hospital stay was shorter for device closure than for surgical repair. Appropriate patient selection is an important factor for successful device closure. Transcatheter closure of secundum ASD using the ASO is a safe and effective alternative to surgical repair.

The role of patent foramen ovale (PFO) in patients with cryptogenic stroke (stroke of unknown cause) remains controversial, although an association seems likely in younger patients with atrial septal aneurysms and PFO. The mechanism of cryptogenic stroke in these patients is presumed to be paradoxical embolism via right-to-left shunt across the PFO. The available options for treatment include medical therapy with antiplatelet or anticoagulant therapy or closure of the PFO surgically or with use of transcatheter PFO closure devices. We describe 2 cases of bilateral device thrombosis associated with use of a transcatheter PFO closure device (CardioSEAL). To our knowledge, only 1 other case of thrombosis associated with use of this device has been reported.

Patent foramen ovale (PFO) is implicated in platypnea-orthodeoxia, stroke and decompression sickness (DCS) in divers and astronauts. However, PFO size in relation to clinical illness is largely unknown since few studies evaluate PFO, either functionally or anatomically. The autopsy incidence of PFO is approximately 27% and 6% for a large defect (0.6 cm to 1.0 cm). A PFO is often associated with atrial septal aneurysm and Chiari network, although these anatomic variations are uncommon. Methodologies for diagnosis and anatomic and functional sizing of a PFO include transthoracic echocardiography (TTE), transesophageal echocardiography (TEE) and transcranial Doppler (TCD), with saline contrast. Saline injection via the right femoral vein appears to have a higher diagnostic yield for PFO than via the right antecubital vein. Saline contrast with TTE using native tissue harmonics or transmitral pulsed wave Doppler have quantitated PFO functional size, while TEE is presently the reference standard. The platypnea-orthodeoxia syndrome is associated with a large resting PFO shunt. Transthoracic echocardiography, TEE and TCD have been used in an attempt to quantitate PFO in patients with cryptogenic stroke. The larger PFOs (approximately ≥4 mm size) or those with significant resting shunts appear to be clinically significant. Approximately two-thirds of divers with unexplained DCS have a PFO that may be responsible and may be related to PFO size. Limited data are available on the incidence of PFO in high altitude aviators with DCS, but there appears to be a relationship. A large decompression stress is associated with extra vehicular activity (EVA) from spacecraft. After four cases of serious DCS in EVA simulations, a resting PFO was detected by contrast TTE in three cases. Patent foramen ovales vary in both anatomical and functional size, and the clinical impact of a particular PFO in various situations (platypnea-orthodeoxia, thromboembolism, DCS in underwater divers, DCS in high-altitude aviators and astronauts) may be different.

Sitting position during neurosurgery is discussed because the risk of venous air embolism and paradoxical air embolism is increased. Preoperative transoesophageal echocardiography is proposed to screen patients with patent foramen ovale to avoid them for the sitting position. This work reported 2 patients in conflict with this screening. It is discussed the physiological principles governing the paradoxical air embolism and the actual recommended monitoring for this position.

We present a case of persistent primitive olfactory artery (PPOA) diagnosed with the aid of magnetic resonance (MR) angiography. The proximal right anterior cerebral artery (ACA) had an extremely long anteroinferomedial course along the ipsilateral olfactory tract, made a hairpin turn posterosuperiorly, and became a normal distal ACA. After reviewing the literature, we considered the proximal segment of this anomalous ACA to be a PPOA. To our knowledge, this is the first report of a patient with this anomalous ACA diagnosed with MR angiography.

This review shows that clinical signs cannot be relied on to reveal left-to-right ductal shunting in the preterm. Echocardiography can define many of the haemodynamic features, including occult ventricular dysfunction, and categorize shunts into small, moderate or large, without defining "significance" which is multifactorial. Large shunts have an elevated left atrial:aortic root ratio (>1.3:1 or >1.5:1, depending on fluid and diuretic policy) a ductal diameter >1.4 mm and retrograde diastolic flow in the descending aorta exceeding 30% of the antegrade flow. The neonatal clinician and echocardiographer is reminded to remain wary of occult congenital heart disease, particularly before closing the ductus.

Patent ductus arteriosus (PDA) is a common problem in very preterm infants. It results in a significant left-to-right shunt and an increase in left ventricular output. Pulmonary compliance can be reduced. Systemic effects result from the diastolic steal and retrograde diastolic blood flow. Randomized controlled trials of PDA closure fall into three groups: (i) prophylactic treatment in the first 24 h, (ii) pre-symptomatic treatment on ultrasound evidence of a PDA or the first clinical signs and (iii) treatment when it becomes haemodynamically significant. Prophylactic treatment with indomethacin reduces the incidence of intraventricular haemorrhage. All the trials have a decreased need to treat a subsequent PDA in the treatment group. There are no other improvements in outcome, without any change in mortality, bronchopulmonary dysplasia, necrotizing enterocolitis or retinopathy of prematurity. Clinical decisions on the treatment of the ductus should be individualized and based on the gestation of the baby, the respiratory condition and the size of the ductal shunt.

a frequence de l’embolie paradoxale est probablement sous-estimee (1). Les criteres diagnostiques ne sont pas consensuels mais reposent sur un faisceau d’arguments (2, 3) : – une thrombose veineuse profonde (TVP) ou une embolie pulmonaire (EP) ; – une communication anormale entre les circulations droite et gauche ; – une embolie systemique ; – un gradient de pression favorisant un shunt droit-gauche. Evoquer ce diagnostic est important car il impose une anticoagulation prolongee ainsi que peut-etre une fermeture du foramen ovale patent (FOP) en cas de recidive (4).

Three patients presented to our intensive care unit over a 3-yr period with profound hypoxia resulting from acute right-to-left inter-atrial shunt (RLIAS). Patient 1 was a 67-yr-old male with an atrial septal defect who became hypoxic and developed the rare sign of platypnoea following elective repair of an abdominal aortic aneurysm (breathlessness made worse when upright and relieved by lying flat). Patient 2 was a 38-yr-old female who developed platypnoea and hypoxia secondary to a patent foramen ovale (PFO) and pericardial effusion. Patient 3 was a 46-yr-old male with a PFO who developed hypoxia without platypnoea because of multiple pulmonary emboli following right hemicolectomy. These case reports illustrate the need to consider RLIAS as a cause of hypoxia of sudden onset. Early use of bubble contrast echocardiography is indicated.

Objective: To describe the relationship among ductal shunting, estimated pulmonary blood flow, and pulmonary hemorrhage in very preterm infants. Study design: A total of 126 babies born before 30 weeks' gestation (median gestation 27 weeks, range 23 to 29 weeks) underwent echocardiography at 5, 12, 24, and 48 hours of age; measurements included right and left ventricular output, superior vena cava flow, and color Doppler diameter of any ductal shunt. Pulmonary blood flow was derived from the sum of right ventricular output and estimated ductal shunt flow. Results: Twelve (9.5%) babies had a pulmonary hemorrhage at a mean age of 38 hours. Compared with the rest of the cohort, these 12 babies were less likely to have had antenatal steroids (59% vs 90%) and were less mature (26 weeks vs 27 weeks). At the echocardiogram closest to the pulmonary hemorrhage, 11 (92%) of the 12 babies had a significant patent ductus arteriosus >1.6 mm in diameter (median 2 mm, range 0.7 to 2.4 mm), and the median pulmonary blood flow was 326 mL/kg/min (range 210 to 598 mL/kg/min). These measurements were significantly higher than those found in the rest of the cohort in the same period (median duct diameter 0.5 mm [range 0 to 2.9 mm], median pulmonary blood flow 237 mL/kg/min [range 107 to 569 mL/kg/min]). At 5-hour echocardiography the babies with pulmonary hemorrhage had significantly larger diameter ducts but similar pulmonary blood flow. Conclusions: Pulmonary hemorrhage in preterm babies is associated with significant ductal shunting and high estimated pulmonary blood flow. (J Pediatr 2000;137:68-72)

Accurate evaluation of secundum atrial septal defect (ASD) is important for the success of transcatheter closure. The purpose of this study was (1) to describe a quantitative evaluation by transthoracic (TTE) and transesophageal (TEE) echocardiography of the various morphologic features of ASD that might be important for patient selection for transcatheter closure, and (2) to assess the reliability of these 2 methods by surgical findings. Preoperative TTE and TEE were used to evaluate the ASD of 27 children undergoing surgical closure. Measurements included the diameters of ASD and the atrial septal lengths in the transverse and longitudinal axis, and the width of the superior, inferior, anteroinferior, and posterior septal margins. The shape and location of the ASD and the adequacy of the septal margins for anchoring occluding devices were determined. Echocardiographic data were compared with corresponding surgical measurements. No significant difference occurred in the means of all the parameters measured with the 3 methods, except for the transverse ASD diameter (p

Resume Un garcon de deux ans et demi a ete hospitalise en chirurgie infantile pour un hepatoblastome requerant une hepatectomie. Celle-ci a necessite la mise en place d'un catheter veineux central, pose au bloc operatoire, par voie sus-claviculaire sans aucune difficulte. La radiographie de thorax de controle a montre une projection du catheter en regard du bord gauche du cœur. Le diagnostic de veine cave superieure gauche persistante a ete alors suspecte sur l'aspect de la courbe de pression veineuse centrale. La radiographie postoperatoire du thorax a confirme le diagnostic. Le catheter a ete laisse en place jusqu'au cinquieme jour, sans aucune complication. Une description de la formation du systeme veineux cave superieur et de ses anomalies est rappelee.

Ultrasound guided patent foramen ovale (PFO) closure has traditionally utilized transoesophageal echocardiography (TOE) under general anaesthesia. Some centres use fluoroscopic guidance alone to facilitate day case PFO closure. Intracardiac echocardiography (ICE) is performed via femoral vein access using an 11 Fr sheath providing accurate guidance without the necessity for general anaesthesia. The safety and feasibility of PFO closure using ICE guidance as a day case procedure have not been documented. We present a consecutive series of patients undergoing planned day case PFO closure under ICE guidance with transthoracic echocardiogram (TTE) follow up. Patients excluded from day case PFO closure were those with early pregnancy or unfavourable social circumstances. 53 consecutive adult patients (44.2+/-11.0 years; 24 males) were planned for day case PFO closure facilitated by ICE. Referral indications were stroke or TIA (n=39), peripheral embolism (n=6), decompression sickness (n=7) and severe migraine (n=1). All 53 patients underwent ICE, with 9/53 (17%) having an atrial septal aneurysm. In 5 patients no PFO was found. In the remaining 48 patients, PFO closure was achieved using the HELEX occluder (n=47) or the Amplatzer device (n=1). Mean procedure and fluoroscopy times were 31.0+/-12.4 and 5.3+/-3.9 min respectively. One patient failed same day discharge due to groin haematoma. There were no other complications. At 3 month follow up, 45/48 (94%) had no residual shunt, with 3 patients having small residual shunts on colour flow Doppler. In conclusion, percutaneous PFO closure as a day case procedure is safe and feasible when facilitated by ICE.

Background . The earliest open-heart operations were performed employing the thoracotomy approach. Over the years, median sternotomy has become the routine way of approaching the heart. However, lately there has been progressive enthusiasm in minimally invasive techniques for accessing the heart. We present our technique of correction of congenital heart defects employing the limited posterior thoracotomy approach. Methods . From June 1997 to April 1998, 27 patients underwent correction for various intracardiac defects without any mortality. There were 19 ostium secundum defects, with or without other associated anomalies. There were six sinus venosus defects with partial anomalous pulmonary venous connections. Two patients had perimembranous ventricular septal defects, while 2 patients had partial atrioventricular defects. In 2 other patients, pulmonary stenosis was repaired, using pulmonary valvotomy in 1 patient, whereas the other patient required short transannular patch. Results . The median age was 7 years and the median weight was 20 kg. The median skin-to-skin time was 260 minutes. The median bypass time was 63.25 minutes and the median cross-clamp time was 35.0 minutes. All the patients were extubated within 12 hours following surgery and the median ICU stay was 24 hours. Three patients required blood transfusions in the ICU for significant blood loss and the mean chest drainage was 85 cc per 24 hours. None of the patients had phrenic nerve palsies. None of the patients required additional analgesics other than routine ibuprofen or ketorolac tromethamine. Short-term follow-up revealed no functional or physical disability of the thoracic wall and the right arm. All who underwent surgery with this approach were happy with the limited visibility of their scars. Conclusions . Limited posterior thoracotomy offers a viable alternative for midsternotomy and submammary thoracotomy. It has the advantage of a scar in the back that does not impede the future growth of the breast tissue and the pectoralis major. Our approach does not need any new instruments and hence no contraptions are necessary to perform the operation with this approach. Our results have shown satisfactory short-term results and better cosmesis.

Objectives: Results and complications of surgical versus transcatheter treatment of atrial septal defect in the current era are compared. Methods: All consecutive patients with a secundum atrial septal defect and a pulmonary/systemic flow ratio of 1.5:1 or more who presented between May 1997 and June 1998 were enrolled in this study. All patients except those who initially had defects not feasible for interventional occlusion were catheterized to allow interventional closure of the defects. All patients in whom interventional closure could not be performed underwent surgical closure. Results: Sixty-one patients underwent surgery at a median age of 20 years (0.5-74 years) and 61 had the defect closed with an Amplatzer device (AGA Medical Corporation, Golden Valley, Minn) at a median age of 12 years (0.8-77.7 years) (P > .2). Hospital stay in surgically treated patients was 8 days (6-19 days) versus 3 days (3-14 days) in interventionally treated patients (P < .001). Atrial septal defect and shunt sizes were larger in the surgical group ( P < .001). Closure rates in the 2 groups were identical (98%). One patient (68 years) in the surgical group had a perforated duodenal ulcer that necessitated an operation 8 days after closure of the atrial septal defect, and 1 (26 years) had an infected lateral thoracotomy wound necessitating plastic surgery. Embolization of the Amplatzer device to the left ventricle was observed in 1 patient (29 years). The device could be retrieved from the heart, but vascular surgery was required to extract it from the femoral artery. Conclusions: As complete closure rates and complications are identical, but duration of hospital stay is shorter with less morbidity, we prefer implantation of an Amplatzer septal occluder to surgery wherever possible. (J Thorac Cardiovasc Surg 1999;118:674-80)

The aim of this study was to evaluate the diagnostic relevance of 3-dimensional (3D) echocardiography in the assessment of secundum atrial septum defect (ASD2).Twenty-three patients (age 2 to 58 years) with an ASD2 were studied by transthoracic (n = 9) or transesophageal (n = 14) echocardiography for the acquisition of a 3D data set before undergoing surgical repair. Qualitative (location, shape, and structure) and quantitative (largest and smallest anteroposterior and superoinferior diameters) characteristics were analyzed and compared with surgical findings. Intraobserver and interobserver variability were assessed. The gross anatomy of the ASD2, shown by the 3D images, was confirmed by the surgeon in 21 of 23 patients, but the presence of membranous or fenestrated remnants of the valvula foramina ovalis in the defect was not optimally visualized in 7 patients. Three-dimensional echocardiography revealed changes in diameter and shape of the ASD2 during the cardiac cycle. The measured largest and smallest anteroposterior diameters and their intraobserver and interobserver agreement were 274 +/- 12 mm, r = 0. 95 (P.001), r = 0.92 (P.001), and 194 +/- 9 mm, r = 0.96 (P. 001), r = 0.94 (P.001), respectively. The measured largest and smallest superoinferior diameter and their intraobserver and interobserver agreement were 304 +/- 26 mm, r = 0.90 (P.001), r = 0.97 (P.001), and 204 +/- 10 mm, r = 0.83 (P.001), r = 0.84 ( P.001), respectively. The correlation coefficient between 2D and 3D echocardiography for the largest anteroposterior and superoinferior diameter was r = 0.69 (P.001) and r = 0.68 (P =.05), respectively. The correlation coefficient between the measurements from 3D reconstructions and direct surgical measurements was r = 0.20 (P = not significant) and r = 0.57 (P.05), whereas between 2D and surgery was r = 0.50 (P.05) and r = 0.26 (P = not significant).ASD2 has a complex morphology. Three-dimensional echocardiography provides better qualitative and quantitative information on its dynamic geometry, location, and extension as compared with standard 2D echocardiography and might be useful for device selection during catheter-based closure of ASD2.

Summary Objective: Comparison between the diagnostic: accuracy of transcranial Doppler (TCD) relative to transesophageal echocardiography (TEE) In the detection of right-to-left shunt. Design: Detection of right-to-left shunt by TEE with contrast study at rest and during Valsalva maneuver or coughing, and during TCD of the middle cerebral artery using the same contrast test. Detection by TEE was considered positive when air micro-bubbles were detected in the left atrium, and by TCD when a characteristic acoustic signal and typical spike-like interruptions of the Doppler signal detecting the bubbles appeared during the first twelve seconds after the injection. Results: Both examinations were performed in a Medical Intensive Care Unit and in a Nuclear Medicine and Ultrasound Unit in 124 patients (108 with strokes, two with arterial embolisms of upper limbs, two with arterial embolisms associated with pulmonary embolism, eight with chronic respiratory failure, four with ARDS). A right-to-left shunt was detected in 56 patients (45.2%) by TEE and in 64 patients (51.6%) by TCD, TCD sensitivity was 90.9%, specificity 72.2%, positive predictive value 75% and negative predictive value 89.7%. Concordance was found in 82.3% examinations (κ = 0.65). Conclusion: TEE is a semi-invasive examination. TCD is a simple and sensitive method for the detection of a right-to-left shunt. This latter can be performed when it is impossible to perform TEE, and when the examination is limited to search a right-to-left shunt. The main limitation of TCD is that this examination is unable to detect the exact location of the right-to-left shunt.

The AngelWings device is a newer transcatheter device used for closure of secundum atrial septal defects (ASD) and patent foramen ovale (PFO), which consists of a self-centering, 2-disk system. Transesophageal echocardiography (TEE) plays a pivotal role in the deployment of the 2 disks of this device, on the appropriate sides of the atrial septum. The objective of this study is to describe the echocardiographic findings associated with successful deployment of the AngelWings device for closure of ASD and PFO. We evaluated the TEE studies of 70 patients enrolled in 4 United States centers, for closure of ASD and PFO with the AngelWings device. The TEE characteristics of successful and unsuccessful deployments were analyzed. Residual shunts across the atrial septum were assessed by TEE at the end of the procedure, 24 hours later by transthoracic echocardiography, and at 6 months by TEE. The deployment of the device was successful in 65 patients (93%). In the unsuccessful group, ASD size by TEE was larger (13.4 +/- 3.1 vs 8.9 +/- 4.7 mm, p

Platypnaea-orthodeoxia syndrome is usually due to an atrial right-to-left shunt through a patent foramen ovale. We report a case of foramen ovale reopened an unusual circumstance. A 83-year-old patient presenting back pains since a few days was admitted in emergency for dyspnaea and refractory hypoxia. The only way to control her back pains was to stay seated in a kyphoscoliosis position. The imagery explorations pointed out an ascending aortic aneurysm, which compressed the right atrium and distorted the position of the atrial septum relative to caval inflow, when the patient was in her painless position, explaining this gravity-related refractory hypoxia.

A 62-year-old man presented with bilateral thromboembolic occlusion of the lower leg arteries 8 months after closure of a patent foramen ovale with an Amplatzer patent-foramen-ovale occluder (AGA Medical Corporation, Plymouth, MN). Then he developed an acute myocardial ischemia. A left heart catheter revealed thromboembolic occlusion of the right coronary artery, and echocardiography demonstrated a thrombus attached to the device within the left atrium. Cerebral computer tomography showed a new ischemic lesion. In an emergency procedure, the device and the left atrial thrombus were removed, the septal defect was closed, and a coronary artery bypass grafting was performed. The patient was discharged from the hospital in stable condition.

Echocardiography is sometimes performed to identify candidates for coil embolization of patent ductus arteriosus (PDA). Therefore we attempted to determine the validity of color Doppler echocardiography to accurately determine the minimum PDA diameter.We reviewed data from 27 children (median 3.8 years, range 1 to 15 years) with PDAs and measured their minimum ductal diameters by color flow Doppler echocardiography and by angiography. We found that there were significant mean differences between the color Doppler and angiographic measurements (2.7+/-0.8 vs 1.6+/-0.7 mm, P.001) with color Doppler exceeding angiographic measurements byor = 1.0 mm in 15 (56%) of 27 patients. More importantly, color Doppler measured a PDAor = 4.0 mm (usually greater than that recommended for coiling) in 3 (11%) patients in whom the angiographic measurement was4.0 mm. Correlation analysis demonstrated no correlation between the color Doppler and angiographic measurements (r2 = 0.17, P= .04, SEE = 0.2 mm).These data suggest that color Doppler echocardiography often overestimates the true minimum PDA diameter, therefore we recommend that a color Doppler-based recommendation that a PDA is too large for transcatheter coil occlusion not be used exclusively to exclude a patient from this treatment modality.

Background : Pulmonary hypertension and persistent fetal circulation contribute to the high mortality rate associated with congenital diaphragmatic hernia (CDH). Morphological alterations of the pulmonary vasculature in infants with CDH are thought to contribute to exaggerated vasoconstrictor responses to normal vasoconstrictor stimuli. In the pulmonary circulation, hypoxia is a potent vasoconstrictor. Under pathological conditions, hypoxia-induced vasoconstriction may contribute to the development of pulmonary hypertension. Methods : The authors have used the nitrofen-induced model of congenital diaphragmatic hernia in rats to investigate the magnitude of the hypoxic vasoconstrictor response. Congenital diaphragmatic hernias were induced in fetal rats by feeding nitrofen (2,4-dichlorophenyl-p-nitrophenyl ether) to pregnant Sprague-Dawley rats at midgestation. Hypoxia-induced vasoconstriction was measured in isolated, perfused third-generation pulmonary arterioles from normal rats and from rats with nitrofen-induced CDH. Results : The hypoxic vasoconstrictor response was significantly blunted in the pulmonary arterioles of fetal rats with nitrofen-induced (2%±1% vasoconstriction), as compared with the responses observed in normal fetal rats (15%±3% vasoconstriction, P =.004). Conclusion : Blunting of the hypoxic pulmonary vasoconstrictor response may contribute to ventilation-perfusion mismatching in infants with CDH.

Background . Interest in minimally invasive procedures has recently increased because it results in less surgical trauma, decreased patient discomfort, short hospital stay, reduced costs, and better cosmetic appearance. Based on these facts, we have been using the transxiphoid process approach without sternotomy for the correction of atrial septal defects. Methods . From July 1996 to January 1997, the xiphoid process window approach was performed in 10 patients with ostium secundum atrial septal defect. Ages ranged from 6 months to 14 years (mean, 5.3 years). In all patients, extracorporeal circulation was carried out by means of cannulation of the femoral artery and both caval veins and of aortic cross-clamping. Videothoracoscopy was used to improve visualization of the aorta. Results . There were no intraoperative or postoperative complications, and in all but 1 patient, extubation was possible while in the operating room. Conclusions . The xiphoid process window, with no median sternotomy, permitted closure of the atrial septal defects with good results and could be used as a less invasive technique for their correction.

Neonates with a severely restrictive foramen ovale and hypoplastic left heart syndrome (HLHS) present as an emergency. We report the case of a newborn in whom left atrial decompression was successfully achieved by stent implantation into the foramen ovale on the first day of life.