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IMPLICATIONS Centrifugal pumps used to enable atrio-femoral bypass may trigger a change in intra-cardiac shunt flow and increase the risk of morbidity and mortality.

Objectives To conduct a prospective evaluation of the incidence and neonatal outcome of fetuses with persistent right umbilical vein. This condition had traditionally been considered to be extremely rare and to be associated with a very poor neonatal prognosis, but later evidence has raised some doubts about the veracity of these contentions. Methods Between August 1995 and November 1998, 8950 low-risk patients were prospectively evaluated at two medical centers. The sonographic diagnosis of a persistent right umbilical vein was made in a transverse section of the fetal abdomen when the portal vein was curved toward the stomach, and the fetal gall bladder was located medially to the umbilical vein. Results Persistent right umbilical vein was detected in 17 fetuses during the study. Four of them had additional malformations, of which three had been detected antenatally. Conclusions We established that the incidence of persistent right umbilical vein in a low-risk population is 1 : 526. We believe that the sonographic finding of this anomaly is an indication for conducting targeted fetal sonography and echocardiography. When the persistent right umbilical vein is connected to the portal system and other anomalies are ruled out, the prognosis can generally be expected to be favorable.

We report three cases of severe hypoxaemia after ibuprofen administration during a randomised controlled trial of prophylactic treatment of patent ductus arteriosus with ibuprofen in premature infants born at less than 28 weeks of gestation. Echocardiography showed severely decreased pulmonary blood flow. Hypoxaemia resolved quickly on inhaled nitric oxide therapy. We suggest that investigators involved in similar trials pay close attention to pulmonary pressure if hypoxaemia occurs after prophylactic administration of ibuprofen.

The prevalence of a patent foramen ovale (PFO) is ∼30%, and this source of right-to-left shunt could result in greater pulmonary gas exchange impairment at rest and during exercise. The aim of this work was to determine if individuals with an asymptomatic PFO (PFO+) have greater pulmonary gas exchange inefficiency at rest and during exercise than subjects without a PFO (PFO−). Separated by 1 h of rest, 8 PFO+ and 8 PFO− subjects performed two incremental cycle ergometer exercise tests to voluntary exhaustion while breathing either room air or hypoxic gas [fraction of inspired O2 (FiO2) = 0.12]. Using echocardiography, we detected small, intermittent boluses of saline contrast bubbles entering directly into the left atrium within 3 heart beats at rest and during both exercise conditions in PFO+. These findings suggest a qualitatively small intracardiac shunt at rest and during exercise in PFO+. The alveolar-to-arterial oxygen difference (AaDo2) was significantly ( P < 0.05) different between PFO+ and PFO− in normoxia (5.9 ± 5.1 vs. 0.5 ± 3.5 mmHg) and hypoxia (10.1 ± 5.9 vs. 4.1 ± 3.1 mmHg) at rest, but not during exercise. However, arterial oxygen saturation was significantly different between PFO+ and PFO− at peak exercise in normoxia (94.3 ± 0.9 vs. 95.8 ± 1.0%) as a result of a significant difference in esophageal temperature (38.4 ± 0.3 vs. 38.0 ± 0.3°C). An asymptomatic PFO contributes to pulmonary gas exchange inefficiency at rest but not during exercise in healthy humans and therefore does not explain intersubject variability in the AaDo2 at maximal exercise.

Aims The success rate in eliminating a right-left-shunting following percutaneous patent foramen ovale closure is estimated to be > 90%. However up to 10% of patients may have residual shunting following initial closure. Little is known as to the optimum treatment strategy for these patients. We report four cases in which to redo patent foramen ovale closure was possible with a second device. Methods and results At our institution during 2008-2009, 101 patients underwent PFO closure: 81 patients (80%) underwent PFO closure for cerebrovascular events, 12 patients (12%) for migraine with aura, eight patients for systemic embolisation (8%), three patients (3%) for decompression illness and one patient underwent PFO closure for platypnea-orthodexia syndrome. Irrespective of the initial device, redo closure was technically feasible in all cases. All patients had at least a moderate residual shunt evident on echocardiographic evaluation at > 6-month follow-up. The patients in the current study were offered a redo procedure based on the presence of persistent disabling symptoms, as well as increased risk of neurological events, despite adequate antiplatelet therapy and anticoagulation. Conclusions A second percutaneous interatrial septal occluder is feasible in those patients with significant residual shunting following initial closure.

Clinicopathological features were examined in 65 neonates and 8 stillborn infants with pontosubicular necrosis (PSN) compared to 57 neonates and 19 stillborn infants without PSN. Twelve out of 65 neonates with PSN had congenital heart disease and 3 out of 65 neonates showed persistent fetal circulation. On neuropathological examination, the frequency of neonates with PSN who also showed karyorrhetic/eosinophilic neurons in other regions of the brain stem, basal ganglia and thalamus was higher than in controls. The results of this study suggest that acute ischemia is an important underlying pathogenetic factor and PSN occurs in the prenatal as well as postnatal period.

The high fetal pulmonary vascular resistance and pulmonary arterial pressure fall after birth with normal expansion and oxygenation of the lungs. In the condition of persistent pulmonary hypertension (PPH), previously known as persistent fetal circulation, either these physiological changes do not occur or the pulmonary vascular resistance subsequently increases. When the pulmonary arterial pressure exceeds the systemic arterial pressure, right-to-left shunting of blood occurs through the foramen ovale (right to left atrium) and ductus arteriosus (pulmonary artery to thoracic aorta) leading to significant hypoxaemia.

Extracorporeal membrane oxygenation (ECMO) has become an established mode of therapy in many centers for potentially fatal neonatal respiratory failure refractory to conventional therapy. We reviewed the findings of 23 autopsies of patients placed on ECMO therapy during the period from 1988 to 1992 at our institution in order to document the pulmonary histopathologic changes and to correlate such changes with the duration of treatment. Interstitial and intra-alveolar hemorrhages, as well as hyaline membrane formation, were the most common findings during the first few days of therapy. Reactive epithelial hyperplasia (bronchial and type II pneumocytes), squamous metaplasia, and smooth muscle hyperplasia were observed as early as 2 to 3 days after initiation of ECMO therapy. Interstitial fibrosis was noted only after 7 days of ECMO therapy. In three patients treated for 15, 19, and 21 days there was replacement of the terminal airways and alveoli by tall columnar and mucin-producing epithelium. Alveolar and bronchiolar calcifications were noted in seven of the 23 cases in this series. Pulmonary vascular changes were seen in association with persistent fetal circulation, meconium aspiration, and respiratory distress syndrome. These changes are most likely due to the compounded effect of ECMO and the underlying pulmonary insult.

W ITH THE INTRODUCTION of minimally invasive surgical techniques for patent ductus arteriosus (PDA) ligation, the length of hospital stay after the surgery has been substantially reduced.1,2 Regional anesthesia combined with light general anesthesia may further facilitate rapid recovery from surgery and anesthesia, allowing for same-day discharge, or fast-tracking.3 The authors report their initial experience of fast-tracking PDA ligation.

The long-term risk and risk factors for recurrent embolism after percutaneous closure of patent foramen ovale (PFO) were investigated in 152 consecutive patients with presumed paradoxical embolism. During follow-up, the actuarial freedom from recurrent embolism was 95.1% at 1 year, and 90.6% at 2 and 6 years. A residual shunt after percutaneous PFO closure was a predictor for recurrence (RR 5.3; 95% CI 1.3 to 21.0; p = 0.02). Randomized trials comparing medical treatment with percutaneous PFO closure in the prevention of recurrent embolism are in progress.

During the last decade, the Rashkind device (RD) has been used in many countries for the closure of the patent ductus arteriosus (PDA). However, long-term follow-up has not been extensively studied and the need for continuous surveillance is still controversial, in particular in the case of patients with a persistent residual shunt and denying further intervention. Moreover, the risk for infective endarteritis after PDA closure has not been definitively evaluated. The aim of this study was (1) to present long-term data on a large group of patients treated with RD implantation, and (2) to provide data regarding the risk for infective complications and the need for extended follow-up and antibiotic prophylaxis in case of low- and high-risk events.

Aortic dissection was found in a woman, her 2 sons, and 1 of her 3 daughters, and the 3 affected children and a granddaughter had patent ductus arteriosus. The pattern of inheritance of this unique syndrome probably is an autosomal dominant one.

When the results of transesophageal echocardiography was regarded as the gold standard for detecting a patent foramen ovale (PFO) in 136 consecutive patients referred for evaluation of cardiac source of embolism, transthoracic harmonic imaging using saline contrast was superior to fundamental imaging in accuracy for detecting a PFO (sensitivity, 22.5%; specificity [p < 0.05] and sensitivity, 100%; specificity 100%, respectively.)

Several drugs and toxins have been shown to be associated with the development of pulmonary vascular hypertension. Pulmonary hypertension (PH) related to these factors has been classified as primary PH (PPH), currently referred to as idiopathic pulmonary arterial hypertension, because its morphological findings, clinical manifestations, hemodynamic measures, and pathological changes were reported to be similar to those of PPH. In 1998, a clinical classification of PH was proposed, the “Evian classification,” which was updated in 2003. According to the revised clinical classification of PH, this disease with identifiable risk factors is called “pulmonary arterial hypertension (PAH) associated with drugs and toxins”. However, some drugs can be associated with pulmonary veno-occlusive disease (PVOD), persistent PH of the newborn (PPHN), and newly recognized systemic disease, in which the histopathological findings are characterized by pulmonary arteriopathy with medial hypertrophy of muscular and elastic arteries, dilation and intimal atheromas of elastic pulmonary arteries, and right ventricular hypertrophy. Plexogenic arteriopathy can be seen with the exception of PAH of persistent fetal circulation. The identification of drugs and toxins as risk factors for PH poses a great challenge to both the physician and the epidemiologist. Drugs and toxins are categorized according to the strength of their association with PH and the probability of causal role. Table 1 summarizes the drugs and toxins considered in this chapter.

SummaryPatent foramen ovale and obstructive sleep apnoea are frequently encountered in the general population. Owing to their prevalence, they may coexist fortuitously; however, the prevalence of patent foramen ovale seems to be higher in patients with obstructive sleep apnoea. We have reviewed the epidemiological data, pathophysiology, and the diagnostic and therapeutic options for both patent foramen ovale and obstructive sleep apnoea. We focus on the interesting pathophysiological links that could explain a potential association between both pathologies and their implications, especially on the risk of stroke.

Aims To compare the mid-term efficacy and safety of the bioabsorbable BioSTAR(r) device with the non-bioabsorbable CardioSEAL(r) device for percutaneous patent foramen ovale (PFO) closure. Methods and results All 81 consecutive patients who underwent PFO closure with the CardioSEAL(r) or BioSTAR(r) device between June 2003 and July 2008 were included. The presence of a residual shunt (minimal, moderate or large) was measured in both groups at six months follow-up, using contrast transthoracic echocardiography. Forty-four patients (48.4±11.4 years) received the CardioSEAL(r) device and 37 patients the BioSTAR(r) device (47.9±10.7 years). There were no significant differences in short-term complications. Two patients who received the BioSTAR(r) device developed a recurrent transient cerebral ischaemic event. Overall, atrial arrhythmias occurred in 19%, with no difference between both groups. At six months, a residual shunt was present in 29% (27% minimal, 2% moderate) using the CardioSEAL(r) device compared to 28% (17% minimal, 11% moderate) using the BioSTAR(r) device (p=0.18). A predictor for residual shunt could not be found. Conclusions There is no difference in safety and efficacy at six months between the CardioSEAL(r) and BioSTAR(r) device used for PFO closure. However, using the BioSTAR(r) device tends to be associated with a higher percentage of moderate shunting.

Aims: Though the association of patent foramen ovale with cryptogenic stroke in young patients has been known for 20 years, most interventional closure systems are not specifically designed for PFO closure, but instead are adapted from ASD closure systems. We describe the safety, feasibility and efficacy of transcatheter closure of PFO using a dedicated adjustable device specifically designed to overcome some of the pitfalls of PFO occlusion like erosion, left atrial thrombus formation, embolisation, maladaptation to cardiac structures and excessive foreign material deposition. Methods and results: Seventy-two consecutive patients, aged between 20 and 72 years, underwent PFO occlusion using the Premere ® PFO occluder, for the greater part for presumed paradoxical embolism causing cryptogenic stroke or transient ischaemic attack. Forty patients received the 20 mm, while 32 received the 25 mm device. Implantation was successful in all patients. Residual shunt rate, excluding absolutely trivial shunts, was 2.8% at six months on contrast TEE study. Peri- and postprocedural adverse events with some possibility of a causal link to the procedure occurred in six patients. The postprocedural annual recurrence rate (0.99%) was lower than reported in most other series. Conclusions: PFO occlusion using the dedicated Premere ® PFO occluder is effective and safe. The residual shunt rate and post-interventional recurrence rate compares favourably to the literature.

Persistent pulmonary hypertension of the newborn (PPHN) is a potentially life-threatening condition that is characterized by supra-systemic pulmonary vascular resistance causing right-to-left shunting through the ductus arteriosus and/or foramen ovale, leading to a vicious cycle of hypoxemia, acidosis and further pulmonary vasoconstriction. Advances in neonatology including surfactant instillation, high-frequency ventilation, extracorporeal membrane oxygenation and, most importantly, inhaled nitric oxide (INO), have revolutionized the management of PPHN. However, given that INO does not improve oxygenation in a significant proportion (30-40%) of cases, there is an urgent need to consider other therapeutic options for PPHN. The issue is more important for developing nations with a higher PPHN-related health burden and limited resources. This article discusses the evidence about INO in term and preterm neonates in brief, and focuses mainly on the potential alternative drugs in the management of PPHN.

Background— Clinical observations of migraine headache symptoms in patients with a patent foramen ovale (PFO), both of which conditions are highly prevalent, have raised the question of a possible pathophysiological relationship. We sought to evaluate the assumption of an association between migraine headaches and the presence of PFO by use of a large case-control study. Methods and Results— We conducted a case-control study to assess the prevalence of PFO in subjects with and without migraine. Case subjects were those with a history of migraine (diagnosed by neurologists at a specialty academic headache clinic). Control subjects were healthy volunteers without migraine 1:1 matched on the basis of age and sex with case subjects. Presence of PFO was determined by transthoracic echocardiogram with second harmonic imaging and transcranial Doppler ultrasonography during a standardized procedure of infused agitated saline contrast with or without Valsalva maneuver and a review of the results by experts blinded to case-control status. PFO was considered present if both studies were positive. Odds ratios were calculated with conditional logistic regression in the matched cohort (n=288). In the matched analysis, the prevalence of PFO was similar in case and control subjects (26.4% versus 25.7%; odds ratio 1.04, 95% confidence interval 0.62 to 1.74, P =0.90). There was no difference in PFO prevalence in those with migraine with aura and those without (26.8% versus 26.1%; odds ratio 1.03, 95% confidence interval 0.48 to 2.21, P =0.93). Conclusions— We found no association between migraine headaches and the presence of PFO in this large case-control study.

Aims: To summarise our experiences of device closure of residual shunt after transcatheter closure of patent foramen ovale (PFO). Methods and results: Since October 1997 implantation of a second closure device was attempted in 40 patients with a moderate or large residual shunt after implantation of a PFO closure device. The mean age of the patients was 51 years. Implantation of a second closure device was technically successful in 39/40 patients (98 %). The following devices were implanted: Premere™ (n=20), Amplatzer ® PFO (n=13), STARFlex ® (n=4), Helex™ (n-1), Angelwings (n=1). During a mean follow-up of 36±29 months complete closure was achieved in 27 patients (69 %). The remaining shunt was small in nine patients, moderate in one and large in two. One patient with a moderate and one patient with a large residual shunt received a third device. The third patient was sent to surgery. One patient died 21 days after implantation of a third closure device due to acute pericardial tamponade. No other complications occurred. Conclusions: Transcatheter implantation of a second closure device after PFO closure is feasible. Complete closure can be achieved in the majority of patients.

As there is no other measurement of right ventricular preload, central venous pressure (CVP) measurement provides unique and important haemodynamic information. CVP is not measured routinely in neonatology and there is a shortage of data in the ventilated neonate. CVP was measured in 62 ventilated neonates. Thirteen had respiratory disorders (28-42 weeks' gestation, birth weight 860-4390 g) and 49 had congenital heart disease (birth weight 1600-4500 g, age 0.5-30 days). Data from other case reports are also presented. In the babies with respiratory distress, a value of zero was associated with clinical evidence of hypovolaemia and negative values, common in the unventilated neonate, did not occur in those who were ventilated. Values over 7 mm Hg were found in babies with evidence of myocardial dysfunction or persistent fetal circulation but were also found with transmitted high intrathoracic pressure, such as with pneumothorax. In the babies with congenital heart disease, values mostly lay between 4 and 8 mm Hg. Values outside this range, particularly above 8 mm Hg, were usually associated with profound metabolic acidosis, suggesting circulatory failure. While the main use of CVP measurement is in trend analysis, this report suggests that single measurements can be of value, though correct interpretation will depend on the context in which they are made.

The outcome of fetuses with diaphragmatic hernia (CDH) has been reported to be related to the severity of lung hypoplasia. As an index of pulmonary hypoplasia, we attempted to measure the lung-thorax transverse area ratio (L/T) using ultrasonic echography in eight fetuses with left-sided CDH. Two cases with L/T more than 0.28 (controls: 0.52±0.04) were transported postnatally and recovered after early operation without episodes of persistent fetal circulation. Elective surgical repair was performed in six infants immediately after cesarean delivery at 35–37 weeks' gestation. In three cases with L/T between 0.21 and 0.24 who recovered with no complications, surgical reduction of the abdominal organs improved arterial blood gases and high-frequency oscillation ventilation (HFOV) was fully effective for respiratory management. In three with L/T between 0.11 and 0.17, extracorporeal membrane oxygenation (ECMO) was required from the 1st to the 12th postoperative day despite HFOV. Although two infants died of combined cardiovascular anomalies and airway bleeding caused by prolonged HFOV, respectively, one infant with minimal L/T survived. Measurement of L/T may help to predict the outcome of fetuses with CDH and to determine the indications for various treatments including immediate operation after cesarean delivery, HFOV, and ECMO.

Meconium-stained amniotic fluid occurs in approximately 12% of live births. In approximately one third of these infants meconium is present below the vocal cords. However, meconium aspiration syndrome develops in only 2 of every 1000 live-born infants. Ninety-five percent of infants with inhaled meconium clear the lungs spontaneously. Recent investigations have suggested that a reexamination of our assumptions about the etiology of meconium aspiration syndrome is in order. Several authors have provided evidence that support the hypothesis that it is not the inhaled meconium which produces the primary pathologic condition of meconium aspiration syndrome but rather it is fetal asphyxia that is the etiologic agent. Asphyxia in utero produces pulmonary vasospasm and hyperreactivity of the pulmonary vessels. With severe asphyxia the fetal lungs undergo pulmonary vascular damage with pulmonary hypertension. The damaged lungs are then unable to clear the meconium. In the most severe cases there is right-to-left shunting and persistent fetal circulation with subsequent fetal death. The incidence of meconium aspiration may thus be essentially unaffected by current obstetric and pediatric interventions at birth. For the asphyxiated or distressed infant we recommend suctioning at birth and tracheal intubation. In the healthy fetus observation may be sufficient.

Surgical repair of atrial septal defect in adults reduces right ventricular and right atrial diameters and volumes, and improves left ventricular filling.

We describe an uncommon association of deletion 22q11 in a patient with Klinefelter syndrome. Even though congenital heart defects (CHD) are not associated with Klinefelter syndrome, further investigation of this patient with patent ductus arteriosus showed a microdeletion of chromosome 22q11.2. While this finding may be coincidental, it is important to further evaluate patients when the clinical features are suggestive of a secondary abnormality.

A case of patent ductus venosus (PDV) presenting intrapulmonary shunting is described. Although retrograde venography of ductus venosus showed few intrahepatic branches, banding of PDV resulted in increased intrahepatic portal branches and disappearance of symptoms 10 months after the operation. Banding of the ductus venosus may be effective in PDV even with hypoplastic intrahepatic portal system.

A successful repair of anomalous left pulmonary artery from the ascending aorta via a left ductus arteriosus in a 1 kg baby is reported. Repair was performed at an early age to avoid pulmonary hypertension and left pulmonary artery occlusion. Utilizing the right ductus to perfuse the right lung, surgery was performed without cardiopulmonary bypass.

The platypnea-orthodeoxia syndrome is a rare clinical presentation. The differential diagnosis is short and includes cardiac, hepatic, and pulmonary causes, with right-to-left intracardiac shunt being the most common. A secondary process is usually present in conjunction with an intracardiac shunt in order for platypnea and orthodeoxia to develop. We present a 63-year-old man in whom the platypnea-orthodeoxia syndrome was associated with a patent foramen ovale and symptoms were manifested by the subacute development of pulmonary arterial hypertension mediated by pulmonary tumor emboli. On postmortem examination, the patient had an underlying poorly differentiated lung adenocarcinoma. This case provides a concise review of the platypnea-orthodeoxia syndrome and pulmonary tumor emboli and stresses the importance of looking for a secondary process in conjunction with an intracardiac shunt in establishing the underlying diagnosis.

Definition Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome resulting from maladaptation to extrauterine life without a sustained normalization of pulmonary vascular resistance (PVR). PPHN is characterized by an elevation in PVR with resultant hypoxemia due to right-to-left shunting at the ductal and/or atrial level. PPHN may be confused with, or may coexist with, congenital heart disease: PPHN is frequently idiopathic but may be associated with respiratory failure/alveolar hypoxia (e.g., meconium aspiration syndrome, congenital pneumonia, sepsis, birth asphyxia, respiratory distress syndrome (RDS)), lung hypoplasia (e.g., congenital diaphragmatic hernia, alveolar capillary dysplasia) and maternal medication (e.g., late gestational use of selective serotonin reuptake inhibitors (SSRI); see Table 3.8). Syndrome Persistent fetal circulation (PFC) is used rarely. Epidemiology Incidence 0.67/1000 to 1.9/1000 live births. The risk of PPHN is about sixfold increased in newborns whose mothers used antidepressants of the SSRI class (e.g., Prozac®) after 20 weeks of gestation, with an overall incidence of approx. 6 to 12 per 1000 exposed women. However, the latter risk increase is based on a retrospective case controlled study with a very small effect size, i.e., 99% of women exposed to SSRI will have a baby not affected by PPHN. The incidence of “idiopathic” PPHN in neonates with Down syndrome (trisomy 21) is high (≈12/1000), but in the absence of associated conditions there is resolution of PPHN. Etiology/pathophysiology See also p. 63 “Postnatal cardiopulmonary adaptation” […]

Among patients with a patent foramen ovale (PFO) and a prior cryptogenic ischemic stroke or TIA, the absolute and relative risk of recurrent events is unclear.We conducted a systematic review and meta-analysis of clinical studies in any language published up to February 2008. We included studies reporting original data on recurrent cerebrovascular events in patients with prior cryptogenic stroke or TIA and PFO. Two authors independently extracted data and evaluated study quality.We identified 15 eligible studies, four with a non-PFO comparison group. In these four studies, the pooled relative risk (RR) for recurrent ischemic stroke or TIA in patients with vs without a PFO was 1.1 (95% confidence interval [CI] 0.8 to 1.5). For ischemic stroke, the pooled RR was 0.8 (95% CI 0.5 to 1.3). We tabulated the absolute rate of recurrent events in all 15 studies. The pooled absolute rate of recurrent ischemic stroke or TIA in patients with PFO was 4.0 events per 100 person-years (95% CI 3.0 to 5.1) while the rate of recurrent ischemic stroke was 1.6 events per 100 person-years (95% CI 1.1 to 2.1).In medically treated patients with prior cryptogenic stroke, while the absolute rate of recurrent events is variable, available evidence does not support an increased relative risk of recurrent ischemic events in those with vs without a patent foramen ovale. Patent foramen ovale closure in these patients cannot be recommended until the results of ongoing clinical trials are reported.

Background and Purpose— Patent foramen ovale (PFO) is significantly associated with cryptogenic stroke (CS). However, even in patients with CS, a PFO can be an incidental finding. We sought to estimate the probability that a PFO in a patient with CS is incidental. Methods— A systematic search identified 23 case-control studies examining the prevalence of PFO in patients with CS versus control subjects with stroke of known cause. Using simple assumptions and Bayes’ theorem, we calculated the probability a PFO is incidental in patients with CS. Random effects meta-analyses estimated the odds ratio (OR) of a PFO in CS versus control subjects in different age populations, with or without atrial septal aneurysms, and were used to summarize across studies the probability that a PFO in CS is incidental. Results— The summary OR (95% CIs) for PFO in CS versus control subjects was 2.9 (CI, 2.1 to 4.0). The corresponding ORs for young and old patients (< or ≥55 years) were 5.1 (3.3 to 7.8) and 2.0 (>1.0 to 3.7), respectively. The corresponding probabilities that a PFO in patients with CS is incidental were 33% (28% to 39%) in age-inclusive studies, 20% (16% to 25%) in younger patients, and 48% (34% to 66%) in older patients. These probabilities were much lower when an atrial septal aneurysm was present. Conclusions— In patients with otherwise CS, approximately one third of discovered PFOs are likely to be incidental and hence not benefit from closure. This probability is sensitive to patient characteristics such as age and the presence of an atrial septal aneurysm, suggesting the importance of patient selection in therapeutic decision-making.

The purpose of this study was to evaluate the efficacy of extracorporeal membrane oxygenation (ECMO) for patients with congenital diaphragmatic hernia (CDH). Ten patients whose mortality was predicted to be more than 80% with previous therapeutics, were selected to use ECMO over the past five years. Patients were placed on ECMO by cannulating the right jugular vein and the right carotid artery. ECMO was started at 34.3 hours after birth on an average, and the average time on ECMO was 96.3 hours. Seven patients (70%) survived, and six out of them are in good health without any sequelae. In particular, all five patients whose pathological condition needing ECMO was persistent fetal circulation (PFC), survived. Therefore we believe ECMO is highly beneficial to overcome the condition of PFC. The survival rate of CDH patients under 24 hours of age in our hospital is 76.2% (16/21 cases) over the last 5 years, and it has improved compared with 57.1% (12/21 cases) before then. The fact that some of the critical patients who were lethal with conventional management, have been successfully treated by ECMO, played a great role in this improvement.

Persistent pulmonary hypertension of the newborn or persistent fetal circulation is a clinical syndrome that is usually apparent within the first 2 days after birth because of the presence of hypoxemia (2;12;19). The syndrome was first described in an abstract by Gersony, Due, and Sinclair (6) in 1969. Two infants were reported who had “RV decompensation, cyanosis and clear lung fields… in the absence of recognizable cardiac, pulmonary, hematologic or CNS disease.” The syndrome has been associated with aspiration of meconium, diaphragmatic hernia, asphyxia, hemorrhage, shock, and maternal infection (4;18). In other cases, there is no clear antecedent event. Despite considerable interest in the problem and a wealth of research related to pulmonary vasoregulation and vascular development in the fetus and newborn, the etiology of the syndrome remains obscure 20 years since its recognition.

A N E U R Y S M OF the ductus arteriosus (DAA) is a relatively rare but potentially fatal condition. The aneurysm may occur either spontaneously or result as a complicat ion after surgical closure of a patent ductus arteriosus (PDA). 1,2 Forty cases of D A A after surgical closure of P D A have been reported to date. 2,3 The diagnosis o f D A A in the pre-1969 period was established at autopsy or during exploratory thoracotomy for suspected lung or mediast inal tumor. 4 D A A is now more frequently diagnosed preoperat ively because of improved diagnostic methods, such as echocardiography 5 and computed tomography. The authors present the successful anesthetic management of a very large false aneurysm of the ductus arteriosus (DA) that presented as a case o f collapse and consolidat ion o f the left lung.

A case of patent foramen ovale opening was observed concomitantly to a defibrillation threshold determination in the setting of an internal cardioverter defibrillator implantation. The subsequent transient right-to-left shunt was confirmed by a peroperative transoesophageal echocontrast study. The underlying mechanism of this incident may be related to a transient reversal of the interatrial gradient, due to the pre-existence of pulmonary hypertension and tricuspid regurgitation, associated with ongoing mechanical ventilation and modifications of intracardiac pressures regimen secondary to the succeeding ventricular tachyarrhythmia and defibrillation. Paradoxical embolism can be an aetiology for neurologic injury during internal cardioverter defibrillator implantation.

We report a case of previously healthy woman who suffered cerebral embolism after delivery. Echocardiography with contrast medium confirmed the patent foramen ovale (PFO). PFO may be a mechanism of paradoxical embolism causing a transient ischemic attack or stroke.

We describe pitfalls of a hitherto undetected patent foramen ovale during the conduct of an off-bypass coronary revascularization. Manipulation of the heart resulted in right-to-left shunt and severe desaturation requiring institution of cardiopulmonary bypass to close the patent foramen ovale and complete the revascularization.

Cyanotic congenital heart disease (CCHD) usually occurs sporadically, but occasionally it is familial without evidence of Mendelian inheritance. The investigators previously reported an association between dominant inheritance of clinically significant atrial shunts (large persistent foramina ovale and small atrial septal defects) and migraine with aura in some families. In 1 family, 4 patients with CCHD were linked by relatives with atrial shunts. The presence of atrial shunts and migraine symptoms was investigated in another family in which 3 members had CCHD. Contrast echocardiography was used to detect whether atrial right-to-left shunts were present in family members. A consultant neurologist, who was blinded to cardiac findings, diagnosed and categorized migraine symptoms. In this family, relatives with atrial right-to-left shunts linked 3 members who had CCHD. There appears to be dominant inheritance of atrial shunts, which is linked to inheritance of CCHD in some families. In conclusion, it is possible that the gene responsible most often causes an atrial shunt but sometimes causes more complex heart disease.

Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arterioles, which, in the absence of effective therapy, progresses rapidly to right heart failure and death. Opening of a patent foramen ovale (PFO) is common in patients with severe pulmonary hypertension (PH), resulting in resistive hypoxemia. We report the case of a 40-year-old woman with idiopathic pulmonary hypertension (iPAH) in New York Heart Association (NYHA) class III to IV, who was admitted in the intensive care unit with hemodynamic compromise and severe hypoxemia due to right-to-left shunt throughout a PFO. Combination therapy initially with inhaled iloprost and sildenafil, and then addition of an endothelin A receptor-selective antagonist (sitaxsentan), resulted in impressive improvement in oxygenation with reversal of the right-to-left shunt and marked improvement in functional class of the patient.

An obese patient with cholelithiasis and acute cholecystitis was scheduled for an emergency laparoscopic cholecystectomy. On preoperative workup, a 5-year-old echocardiogram showed a large patent foramen ovale (PFO) with a right-to-left shunt that was open at rest. Noting both the benefits of laparoscopic surgery and the substantial incidence of PFO in the general population (10% to 30%), the attending and consulting surgeons reasoned that the benefits of laparoscopy greatly exceeded an immeasurably small risk of paradoxical emboli.

Objective: Patent foramen ovale (PFO) has been related to stroke but its existence has not been explained to date. NKX2-5 is the most implicated gene in fetal atrial septation. We studied NKX2-5 with respect to the presence or absence of PFO in stroke patients. Methods: A prospective analysis of NKX2-5 regarding age, gender, PFO, right-to-left shunt (RLS) size and atrial septal aneurysm (ASA) was performed in consecutive stroke patients and in 50 controls. The entire coding region and intron-exon boundaries of NKX2-5 gene were analyzed by PCR and sequencing of DNA from peripheral lymphocytes. Results: One hundred patients participated in the study (mean age 56.5 +/- 12.4 years, 58% males) and PFO was diagnosed in 34% of them by transesophageal echocardiography. RLS was small (12%), moderate (2%) and large (20%). ASA was present in four patients. DNA revealed a novel c.2357G>A change in one PFO patient with cryptogenic stroke. Furthermore, c.182C>T, a mutation previously described in patients with cardiac defects, was detected in two non-PFO women with cryptogenic stroke. None of these changes were detected in our controls. The c.172A>G polymorphism was found in 21% of controls. It appeared more frequently in ASA patients (p = 0.084), in cryptogenic PFO stroke patients (p = 0.097) and in patients with known causes of stroke (p = 0.037). The c.2850C>A polymorphism was also detected in our series with no differences in PFO, RLS size or ASA. Conclusion: Despite the fact that the NRX2-5 could account for the persistence of PFO, mutations of this gene in peripheral blood DNA were barely detected in our study. (C) 2009 Elsevier B.V. All rights reserved.

To evaluate nongated multidetector computed tomography (CT) in the detection of patent foramen ovale (PFO) and atrial septal aneurysm (ASA) in comparison with transesophageal echocardiography (TEE).The study was approved by the institutional review board, and informed consent was obtained from all patients. One hundred five patients (57 men, 48 women; mean age, 53 years) with a recent stroke underwent TEE and multidetector CT on the same day. After injection of a diluted iodinated contrast material, a series of 2.4-mm-thick transverse images centered on the fossa ovalis were acquired in conjunction with release of the Valsalva maneuver. Two independent radiologists considered PFO present if left atrial enhancement was detected visually before enhancement of the pulmonary veins or if an early peak of left atrial enhancement was found at time-attenuation curve analysis.PFO was detected with 98% specificity (95% confidence interval [CI]: 0.91, 0.99). Overall sensitivity was 55% (95% CI: 0.38, 0.70), ranging from 28% for shunts classified as grade 1 at TEE to 91% for those classified as grade 4 at TEE. Interreader agreement was good (kappa = 0.81). Visual assessment was better than time-attenuation curve analysis, which enabled detection of only 15 of the 40 cases of PFO. Multidetector CT depicted only 22% of cases of ASA. The mean effective radiation dose was 2.3 mSv.Nongated multidetector CT can be used to diagnose high-grade shunts through a PFO, with 91% sensitivity and 98% specificity. Thus, PFO detection, in addition to routine CT evaluation of the lungs, could be indicated in patients with unexplained hypoxemia.

Optimal management of patients with PFO and paradoxical embolic events is still debated. Moreover, data from long-term studies on large patient populations are lacking. Aim of the study is to assess immediate and long-term clinical outcome of patients with PFO and paradoxical thrombo-embolic events submitted to transcatheter PFO closure.Only patients with PFO-related transient ischemic attack or stroke underwent PFO closure. Patients were evaluated clinically and echocardiographically at 1, 6 and 12 months after the procedure and yearly thereafter. Primary endpoints were death, recurrent stroke or TIA. Residual right-to left shunt (RLS) was monitored by transthoracic echocardiography (TTE) or transcranial Doppler (TCD) at 6 months'follow-up.202 consecutive patients underwent percutaneous PFO closure for secondary prevention of TE. Device migration was observed in one patient 24 h after the procedure. No cases of procedure-related death or stroke occurred during a median follow-up of 3+/-1.3 years. Three recurrent TIAs were observed within the first 6 months of follow-up. The cumulative estimated probability of recurrent TE-free survival rate after PFO closure was 99% in patientsor=55 years, 84% in patients55 years (p0.05) and 94% and 100% in patients with PFO, with or without atrial septal aneurysm (ASA), respectively (p0.05). Of the 188 (93%) patients submitted to TTE or TCD at 6 months' follow-up, 8 (4%) presented a small RLS.Transcatheter PFO closure is associated with low incidence of in-hospital complications and low frequency of recurrent TE at long-term follow-up.

A 69-year-old woman with an acute pulmonary embolism developed an intracardiac right-to-left shunt, which was diagnosed early on and quantified via biphasic transcardiopulmonary thermodilution curves. With transesophageal echocardiography, a patent foramen ovale and an impressive atrial right-to-left shunt were visualized.

JO N 3126 paradoxical emboli cause spreading depression, several recently published studies showed that the prevalence of MA+ attacks might decrease by 70–80 % after percutaneous PFO closure (for nonmigraine indications) [5]. Unfortunately, these studies were predominantly retrospective, nonrandomized, and conducted in highly selected patients. Recently, the results of the MIST I trial were published. MIST I was a prospective randomized study that evaluated the clinical efficiency of percutaneous PFO closure in patients with MA+. Although a substantial number of patients was included, neither the primary endpoint, complete elimination of migraine, nor the secondary endpoints were achieved [6]. With regard to these results and to the shortcomings of the trial as discussed by John Carroll in a recent editorial in Circulation [7], we performed an early analysis of the FORMAT trial (patent Foramen Ovale closure to Reduce Migraine ATtacks). The FORMAT trial, started in 2005, was a prospective, open-labeled, controlled, and randomized trial to determine whether percutaneous PFO closure with the Intrasept device (CARDIA, Eagan, Minnesota, USA) would influence the frequency of MA+ attacks six months after closure. Only patients with a RLS through a Tom Sarens Luc Herroelen Kristien Van Deyk Werner Budts