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We report a case of an iatrogenic defect in the interatrial septum as a result of mitral valve repair using the transeptal approach.

Cardiac lipomas are rare tumors. They usually remain asymptomatic for a long time and cause angina, arrhythmia, dysfunction of the ventricles or valves, and peripheral embolization during the later stages of development. There is little or no information about right-to-left interatrial shunt with normal pulmonary artery pressure, produced as a consequence of the infiltration of the atrial septum, the right atrial wall, and the myocardium because of the presence of fat in patients with platypnea-orthodeoxia syndrome. We present a patient with this syndrome who was identified through transesophageal echocardiography. The study showed a massive right-to-left shunt without pulmonary hypertension, produced by an important cardiac infiltration of adipose tissue that created a narrow passage in the right atrium, and a redirection of the flow to a patent foramen ovale, explaining the pathophysiology of the syndrome.

Pulmonary vein isolation (PVI) is widely practiced as a means of potentially curing atrial fibrillation (AF). Transeptal puncture is performed for PVI procedures, often two such punctures are performed. We sought to examine the prevalence of medium- to long- term iatrogenic septal defects after such procedures. Thirty-one patients who were undergoing their second PVI procedure were studied with transesophageal echocardiography (TEE) with two-dimensional imaging and color Doppler, examining the fossa ovalis for defects. Mean time from the original PVI to the time of TEE was 35 weeks. Two patients were discovered to have iatrogenic septal defects. The interval between the first PVI procedure and the TEE showing a septal defect was 33.7 weeks in one patient and 14.3 weeks in the other. Iatrogenic septal defects may occur in the medium- to long-term in patients undergoing PVI procedures for AF. The clinical implications of such defects are unknown.

Objective: Paradoxical embolism via a patent foramen ovale (PFO) has been identified as a potential risk factor for ischaemic stroke. Such occurrences are associated with risk factors for deep vein thrombosis (DVT), e. g. pregnancy, immobilisation, and surgery of the lower limbs., Observation: A 54-year-old otherwise healthy female presented with acute onset motoric aphasia and brachiofacial right hemiparesis. The cranial computed tomography showed a left striatal ischaemic infarction. The patient's history revealed a variceal sclerotherapy with polydocanol 0,5% three days prior to the onset of symptoms. Echovist TCM doppler revealed a right-to-left shunt. A patent foramen ovale (PFO) was detected by transesophageal echocardiography. There was no evidence of DVT in bilateral lower-extremity venous duplex ultrasound scanning. Other potential risk factors of stroke including thrombophilia could not be identified. The patient was treated with a high dose regimen of heparin and a further anticoagulation treatment was recommended., Conclusion: This case suggests a probable causal relationship between variceal sclerotherapy and paradoxical embolism resulting in a stroke. Variceal sclerotherapy might be a potential, but rare risk of embolism.

Purpose: To report a case of severe and fatal cardiac complication following pericardiotomy to relieve a malignant tamponade. Right ventricular (RV) failure was responsible for major hypoxemia and for a persistent shunt through a patent foramen ovale. In the absence of pulmonary embolism and coronary occlusion, possible pathophysiologic mechanisms are discussed., Clinical Features: This 53-yr-old patient presented with oropharyngeal carcinoma previously treated by chemotherapy. One month later, he showed clinical and echocardiographic signs of cardiac tamponade. He had a circumferential pericardial effusion with complete end-diastolic collapse of the right cavities. After an emergent pericardiotomy, he rapidly presented severe hypoxemia. Transesophageal echocardiography showed an akinetic and dilated right ventricle, paradoxical septal wall motion and a normal left ventricular function. A contrast study revealed a right-to-left shunt. No residual pericardial effusion was detectable. Pulmonary angiography excluded a pulmonary embolism and the coronary angiogram was normal. Troponin Ic was elevated postoperatively and peaked on day two (3.78 micro g x L(-1)). The patient died of refractory shock with persistent intracardiac shunt and RV akinesia on day nine., Conclusion: Although pulmonary embolism or thrombus of a coronary vessel are the most common causes of prolonged RV failure after pericardiotomy, other mechanisms may be invoked. The possibility is raised that a rapid increase in RV tension may induce the development of muscular injury and impair coronary blood flow, despite a normal coronary angiogram. These could result in a stunned myocardium and opening of a patent foramen ovale. We hypothesize that gradual decompression of a chronic pericardial effusion might be beneficial in patients at risk.

We report a case of postoperative intracardiac shunts across the membranous septum detected by Doppler echocardiography and discuss the anatomical basis for the development of such a complication.

Through advancements in transcatheter technology, a patent foramen ovale (PFO) can now be closed by either a percutaneous or surgical procedure. This report presents a patient who suffered an embolic stroke secondary to a PFO. The PFO was successfully closed surgically, but 7 years later the foramen ovale was found to be widely patent. The patient underwent successful percutaneous PFO closure and the foramen ovale remains closed. This case demonstrates that percutaneous PFO closure may be viable in patients in whom surgical closure has not provided long-term closure.

We report a novel palliative treatment to improve refractory cardiogenic shock due to right ventricular ischemia. We performed percutaneous atrial septostomy to create an atrial septal defect, which resulted in immediate right ventricular decompression and increased left ventricular filling associated with prompt and dramatic hemodynamic and clinical improvement., (Copyright 2003 Wiley-Liss, Inc.)

Data on the morphogenesis of atrioventricular septal defect (AVSD) in Down syndrome are lacking to support molecular studies on Down syndrome heart critical region. Therefore, we studied the development of complete AVSD in human embryos and fetuses with trisomy 21 using 3-dimensional graphic reconstructions and immunohistochemical markers. Eight trisomic hearts with AVSD and 10 normal hearts, ranging from 5 to 16 weeks' gestation, were examined. In AVSD, the muscular septum primum and venous valves develop normally, and the size and histology of the nonfused endocardial cushions also appear normal. However, the mass of extracardiac mesenchyme (vestibular spine), located at the dorsal mesocardium, is reduced and does not protrude ventrally along the right wall of the common pulmonary vein. As a result of this, the muscular septum primum and the right pulmonary ridge are seen as 2 separate septa that attach to the inferior endocardial cushion. Both the muscular septum primum and the superiorly fused venous valves (septum spurium) converge and are capped by a small rim of mesenchyme, which forms the roof of the persisting ostium primum and connects to cushions and the reduced vestibular spine. At 7 weeks, ventricular septation in AVSD is comparable to 5 to 6 weeks of normal cardiac development. At later stages, the septum spurium forms the anterosuperior limbus of the septum secundum and the mesenchymal cap becomes the bridging tendon that connects the bridging leaflets. Therefore, reduced expansion of the vestibular spine derived from the dorsal mesocardium appears to play an important role in the development of AVSD in Down syndrome.

This case illustrates the reopening of foramen ovale in a young patient with chronic pulmonary hypertension caused by bronchiectasis and chronic pulmonary fibrosis, which resulted in a prominent right-to-left shunt and severe hypoxia. Her clinically unsuspected right-to-left shunt was discovered during ventilation-perfusion scan, which was performed for the evaluation of pulmonary embolism. She had common variable immune deficiency, a primary immunodeficiency disease in which B-lymphocytes produce few or no antibodies. Most patients with this syndrome have an intrinsic defect in their B-lymphocytes that results in reduced immunoglobulin production. In these patients, recurrent respiratory tract infections are common and may result in chronic lung disease, fibrosis, particularly bronchiectasis (20-30%) and even cor pulmonale as happened in our patient [J. Clin. Immunol. 9 (1989) 22-33.].

Background: The posttraumatic fat embolism syndrome (FES) is characterized by petechiae and pulmonary and cerebral dysfunction. A patent foramen ovale (PFO) could worsen the prognosis of FES by allowing larger emboli to reach the systemic circulation. Transcranial Doppler ultrasonography can be used to diagnose and monitor cerebral microembolism in FES., Objective: To describe a case of successful percutaneous closure of PFO in a patient with posttraumatic FES with excellent clinical outcome., Patient and Methods: A 17-year-old girl presented with a posttraumatic long-bone fracture complicated by typical severe FES. Transcranial Doppler disclosed multiple microembolic signals over both middle cerebral and basilar arteries. A large PFO was diagnosed by transesophageal echocardiogram. A brain magnetic resonance image with diffusion-weighted sequences showed multiple bilateral areas of abnormal diffusion in watershed territories. Percutaneous PFO closure with a buttoned device was successfully performed., Results: Closure of PFO was associated with marked reduction in the number and intensity of microembolic signals. Subsequent surgical repair of the fracture with the patient under transcranial Doppler monitoring was uneventful. There was excellent correlation between clinical course and microembolic signal load by transcranial Doppler., Conclusions: Cerebral fat embolism after long-bone fractures can be detected in vivo and monitored over time with the use of transcranial Doppler techniques. If a PFO is present, its closure before surgical manipulation of the fracture is feasible and could have important protective effects against massive systemic embolization.

Transseptal perforation using radiofrequency energy was performed successfully in a patient with congenital heart disease and a thickened interatrial septum. This was followed by balloon dilatation of the atrial septal defect. Radiofrequency is presented as a alternative to standard transseptal needle puncture. Cathet Cardiovasc Intervent 2001;54:83-87., (Copyright 2001 Wiley-Liss, Inc.)

We describe residual atrial septal defects in 3 patients who had previous surgical repair. The residual defects were the sinus venosus type near the orifice of the inferior vena cava. Preoperative and intraoperative transesophageal echocardiography may aid in the detection and facilitate the successful repair of these defects.

Deviation of atrial septum primum (DASP) is an unusual anatomic finding in patients with normal left atrioventricular valve size. To better describe this anomaly, we reviewed our echocardiographic database from January 1987 to May 1999. Twenty-four patients had DASP and a normal-sized left atrioventricular valve. Six had inferior DASP with common atrioventricular canal. Of these, 3 had evidence of left atrial outlet obstruction. Five survived surgical intervention with follow-up of 24 +/- 14 months. Seventeen patients had superoposterior deviation with the right pulmonary veins draining anomalously to the right atrium. Only 8 of 17 underwent surgical resection; none of the 17 have evidence for late development of obstruction. One patient had both inferior deviation and superoposterior DASP and underwent early successful repair. DASP occurs in patients with normal left atrioventricular valve size. Inferior DASP can result in left atrial outlet obstruction and requires surgical intervention. Superoposterior DASP carries less physiologic consequence. Echocardiography can distinguish this anomaly from other forms of left atrial obstruction.

Hypoxemia related to right-to-left shunting through a patent foramen ovale (PFO) is not rare. It can be observed in correlation with a specific situation such as pneumonectomy and can occur even with normal pulmonary pressure. This article reports the experience of 12 patients in which a transcatheter closure with the CardioSeal device was done successfully. Clinical improvement is often obtained, despite incomplete occlusion of the defects. The author demonstrated that transcatheter closure of PFO can be performed safety and should be considered as an efficient alternative to surgery in cyanotic patients with PFO.

Background: Recent advances in clinical, pathological, and genetic aspects of atrioventricular septal defects (AVSD) have set the stage for epidemiologic investigations into possible risk factors. Previous analyses of the total case group of AVSD included complete and partial subtypes without analysis of the subsets., Methods: To address the question of possible morphogenetic heterogeneity of AVSD, the Baltimore-Washington Infant Study data on live-born cases and controls (1981-1989) was reanalyzed for potential environmental and genetic risk-factor associations in complete AVSD (n = 213), with separate comparisons to the atrial (n = 75) and the ventricular (n = 32) forms of partial AVSD., Results: Complete and ventricular forms of AVSD had a similar proportion of isolated cases (12.2% and 15.6%, respectively, without associated extracardiac anomalies) and high rates of Down syndrome, whereas the atrial form of partial AVSD included 55% isolated cases. Trisomy 18 occurred in 22% of infants with the ventricular form, compared with <2% in the other AVSD groups. Analysis of potential risk factors revealed further distinctions. Complete AVSD as an isolated cardiac defect was strongly associated with maternal diabetes (odds ratio [OR] = 20.6; 95% confidence interval [CI] =5.6-76.4) and also with antitussive use (OR = 8.8; CI = 1.2-48.2); there were no strong associations other than maternal age among Down syndrome infants with this type of heart defect. Isolated cases with the atrial type of partial AVSD were associated with a family history of heart defects (OR = 6.2; CI = 1.4-24.4) and with paternal occupational exposures to ionizing radiation (OR = 5.1; CI = 1.4-27.4), but no risk factors were associated with Down syndrome. There were no significant associations of any risk factors in the numerically small subsets of isolated and Down syndrome cases with the ventricular form of partial AVSD., Conclusions: These results indicate a similar risk profile of complete AVSD and the ventricular type of partial AVSD, with a possible subset of the latter due to trisomy 18. Maternal diabetes constituted a potentially preventable risk factor for the most severe, complete form of AVSD., (Copyright 2001 Wiley-Liss, Inc.)

We present a case of transcatheter closure of an atrial baffle leak with significant systemic to pulmonary atrium shunt in a patient late after Mustard operation and pulmonary valvotomy for transposition of the great arteries. This procedure alleviated the need for reoperation in a high-risk symptomatic patient. Cathet. Cardiovasc. Intervent. 51:305-307, 2000., (Copyright 2000 Wiley-Liss, Inc.)

Objective: To report the surgical correction of complete atrioventricular septal defect with tetralogy of Fallot (AVSD-TOF)., Methods: Six consecutive patients aged 3 - 9 years underwent correction of complete AVSD-TOF. The two-patch technique for atrioventricular septal defect was used. The ventricular septal defect was closed through a right ventriculotomy and right atriotomy in each case. The commissure between the superior and inferior bridging leaflets of the left portion of the common atrioventricular valve was closed in each patient. RVOT obstruction was relieved by a transannular autologous pericardium with monocuspid valve., Results: Postoperative complications included respiratory failure in 1 patient, low cardiac output syndrome in 1, and MOF in 1. There was one mortality because of MOF in the early postoperative period. Five survivors were followed up from 6 months to 5.5 years (mean 2.3 years). There was no late mortality and only one patient had mild left atrioventricular valve regurgitation. NYHA cardiac function was class I in 4 patients and class II in 1., Conclusions: AVSD-TOF can be corrected using the two-patch technique and closure of the ventricular septal defect through a combined approach using a right ventriculotomy and right atriotomy. Routine closure of the commissure of the left portion of the atrioventricular valve results in a low incidence of regurgitation. Good functional result can be achieved in most patients postoperatively.

The authors describe an acquired atrioventricular septal defect that has resulted from a blunt chest trauma. Besides being an uncommon traumatic heart injury, this case has the particularities of the non-involvement of other adjacent anatomical structures and the long delay between the accident and the occurrence of the myocardial rupture.

We studied the value of routine neonatal electrocardiography (ECG) in the 1st 48 hours of life to diagnose congenital heart disease in 37 neonates with Down syndrome. Twenty-four infants had no clinical evidence of congenital heart disease, had normal ECGs and normal cardiac anatomy on echocardiography. Thirteen children (35.2%) had congenital heart disease. The ECG was normal in seven infants with congenital heart disease: four with atrial septal defect (ASD), two with tetralogy of Fallot (TOF) and one with ventricular septal defect (VSD). A left QRS axis deviation was found in six infants: five with complete atrioventricular septal defect (AVSD) and one with VSD and mitral valve prolapse. However, only the five infants with complete AVSD had a superior QRS axis. Although neonatal ECG detected the presence of complete AVSD in neonates with Down syndrome, it missed an equal number with other heart defects. Echocardiography remains the way reliably to diagnose or exclude congenital heart disease in these infants.

Objectives: Percutaneous balloon mitral valve commissurotomy (BMC) is an alternative to surgical commissurotomy. Complications following BMC includes mitral regurgitation, iatrogenic atrial septal defect, residual mitral stenosis, and pericardial hemorrhage. This study analyzes the outcomes of surgery following failed BMC for mitral stenosis., Methods: In a series of 298 patients treated with BMC, 53 patients (17.7%) had a complication that necessitated a surgical treatment. Twenty-eight patients needed an immediate surgery before the discharge (group I) and 25 patients were operated on an elective basis (group II)., Results: In group I, 27 patients have been operated and one died before the operation. In 21 patients an acute mitral regurgitation occurred, 3 patients had a residual mitral stenosis, and 3 had a left atrial perforation. The operation consisted of 26 mitral valve replacements, 20 concomitant reparations of iatrogenic atrial septal defect, and one open mitral valve commissurotomy. Operative mortality was 3.7% (1 out of 27). In group II, 25 patients have been operated at a mean 18 +/- 14 months after BMC. In the 25 patients the operation was indicated for significant mitral regurgitation (2 + and more). The operation consisted of 25 mitral valve replacements, 9 concomitant reparations of iatrogenic atrial septal defect, 3 patients had also coronary artery bypasses. The operative mortality was 8% (2 out of 25). The echocardiographic score was similar for both groups, it was 8.4 +/- 2.0 in group I and 8.0 +/- 1.5 in group II (P = NS). Despite these complications following failed BMC, surgery appears a safe procedure with an acceptable mortality.

Objectives: The purpose of the study was to assess the effects of maternal HIV-1 (human immunodeficiency virus) infection and vertically transmitted HIV-1 infection on the prevalence of congenital cardiovascular malformations in children., Background: In the United States, an estimated 7000 children are born to HIV-infected women annually. Previous limited reports have suggested an increase in the prevalence of congenital cardiovascular malformations in vertically transmitted HIV-infected children., Methods: In a prospective longitudinal multicenter study, diagnostic echocardiograms were performed at 4-6-month intervals on two cohorts of children exposed to maternal HIV-1 infection: 1) a Neonatal Cohort of 90 HIV-infected, 449 HIV-uninfected and 19 HIV-indeterminate children; and 2) an Older HIV-Infected Cohort of 201 children with vertically transmitted HIV-1 infection recruited after 28 days of age., Results: In the Neonatal Cohort, 36 lesions were seen in 36 patients, yielding an overall congenital cardiovascular malformation prevalence of 6.5% (36/558), with a 8.9% (8/90) prevalence in HIV-infected children and a 5.6% (25/449) prevalence in HIV-uninfected children. Two children (2/558, 0.4%) had cyanotic lesions. In the Older HIV-Infected Cohort, there was a congenital cardiovascular malformation prevalence of 7.5% (15/201). The distribution of lesions did not differ significantly between the groups., Conclusions: There was no statistically significant difference in congenital cardiovascular malformation prevalence in HIV-infected versus HIV-uninfected children born to HIV-infected women. With the use of early screening echocardiography, rates of congenital cardiovascular malformations in both the HIV-infected and HIV-uninfected children were five- to ten-fold higher than rates reported in population-based epidemiologic studies but not higher than in normal populations similarly screened. Potentially important subclinical congenital cardiovascular malformations were detected.

We report a case of traumatic tricuspid valve regurgitation and late presentation with transient ischemic attacks caused by the presence of a right-to-left shunt through a small atrial septal defect.

Bacterial endocarditis predominantly involves cardiac valves and is associated with many potential complications. Valvular dysfunction resulting from disruption of the structural integrity of valves are not infrequent. This report illustrates a rare case of endocarditis involving the interatrial septum which subsequently produced destruction of the structure resulting in an acquired atrial septal defect.

The authors performed 451 transesophageal echocardiographic (TEE) investigations over a period of three years and four months. Atrial septal aneurysm (ASA) was found in 40 cases. Of these, protrusion of the atrial septum towards the right atrium was observed in 17 cases, whilst oscillation of the atrial septum was noted in 23 cases. ASA was associated with patent foramen ovale (PFO) in ten patients, with type II. ASD in nine patients, with other congenital heart disease in six patients, and with other organic heart disease in eight patients. In three cases either an embolus or a tumor was detected in the left atrium, whilst in four cases with ASA there were no other organic cardiac disorders found. In ten patients there was a history of cerebral embolisation. Of these two had chronic atrial fibrillation, whilst the others had sinusrhythm. Of those who had cerebral embolisation, four patients had PFO, one patient had left atrial and auricular thrombi, whilst in four patients various organic heart problems (ischemic heart disease, left ventricular hypertrophy) were detected. In one patient with ASA there was no other cardiac abnormality detected. The authors conclude that ASA, which is often associated with PFO and ASD (in 25.0% and 22.5% of their cases, respectively) is detected in around eight percent of the patients who undergo TEE. ASA particularly when associated with PFO should be considered as a potential source of cerebral emboli. Indeed, cerebral embolisation occurred in 25% of their patients with ASA. It is recommended, that patients with ASA are treated with acetyl salicylic acid, whilst in patients with ASA and PFO anticoagulant therapy is the treatment of choice. In case of cerebral embolisation, or repeated cerebral ischemic attacks, operative interventions should be considered.

The location, and morphology, of the superior sinus venosus interatrial communication remains contentious. As part of a clinical study, we examined anatomic specimens and echocardiograms so as to clarify the arrangement of the normal atrial septal structures, and compared them with the arrangement found in the superior sinus venosus defect. The pathognomonic diagnostic criterion in the abnormal hearts was overriding of the intact muscular rim of the oval fossa by the mouth of the superior caval vein. This muscular rim is, in reality, a tube of myocardium which encloses a core of extracardiac adipose tissue. Understanding of this anatomic conundrum clarifies the understanding of the structures of both the normal atrial septum and sinus venosus defects.

Study Objectives: The objectives of this study were (1) to assess the prevalence of patent foramen ovale (PFO) by means of contrast transesophageal echocardiography (TEE) in patients with obstructive sleep apnea, and (2) to determine the potential contribution of right to left interatrial shunting to systemic oxygen desaturation following the performance of Valsalva maneuver., Design: Performance of contrast TEE during Valsalva maneuver with simultaneous measurement of systemic arterial oxygen saturation (SaO2) by means of pulse oximetry in patients with obstructive sleep apnea and a control group., Setting: Government teaching hospital, university hospital affiliate., Patients: Study group comprised 48 patients with documented obstructive sleep apnea and 24 control subjects., Interventions: Sleep studies, contrast TEE, Valsalva maneuver, pulse oximetry., Measurements and Results: Thirty-three of 48 patients with obstructive sleep apnea compared with 4 of 24 control patients had a detectable PFO (69% vs 17%; p < 0.0001). All sleep apnea patients had comparable baseline SaO2 regardless of the presence of a PFO (93.9+/-1.7% vs 95+/-1.2%; p=not significant). After performance of a Valsalva maneuver, however, a significantly greater fall in SaO2 was observed in patients with obstructive sleep apnea and PFO compared with patients with obstructive sleep apnea without PFO (-2.4 +/- 1.5% vs -1.3 +/- 0.6%; p=0.007). A statistically significant fall in SaO2 (defined as > 4 SD of recorded SaO2 values after Valsalva maneuver in patients without PFO) was found in one third of patients with sleep apnea and PFO., Conclusion: We conclude that there is an increased prevalence of PFO in patients with obstructive sleep apnea that could contribute to significant hypoxemia after a Valsalva maneuver in approximately one third of these patients.

A previously healthy 64-year-old woman attended our hospital with chest pain, facial edema, and general fatigue. A chest radiograph revealed cardiomegaly, small bilateral pleural effusions, and hilar congestion--findings that improved after early therapy with furosemide and methyldigoxin. A chest radiograph recorded 7 years earlier had revealed no dilation of cardiac shadow. There were no findings suggesting atrial septal defect (ASD) or valvular heart disease. Echocardiography revealed a tumor-like mass adhering to the posterior wall of the left atrium. Color-flow Doppler echocardiography revealed a left-to-right shunt at the atrial level. The Qp/Qs ratio as measured by cardiac catheterization was 2.0. Coronary angiography revealed abnormal dilated arteries from the atrioventricular nodal branch and several feeding arteries from the left circumflex branch. We hypothesized that the left-to-right shunt could be due to the tumor, which extended to the rim of the patent foramen ovale, or to the very small, previously unrecognized, ASD. This patient died 6 months after her first admission and an autopsy was performed. Light microscopic examination of the tumor revealed spindle-shaped fibroblast-like cells arranged in a storiform or fascicular pattern. The immunohistochemical findings were consistent with malignant fibrous histiocytoma (MFH). In the literature, left-to-right shunt at the atrial level has not been reported in patients with cardiac MFH.

This report describes the case of a 67-year-old man in whom atrial right-to-left shunt developed after a right pneumonectomy, leading to dyspnea with severe arterial desaturation. Transcatheter occlusion of the patent foramen ovale was successfully performed using a buttoned device. Review of literature and mechanisms of these atrial right-to-left shunts are discussed.

We present the case of a male infant, born prematurely (at 33 weeks gestation) with macrosomia, disproportionate macrocephaly, facial dysmorphism, short penis and a small umbilical defect. He had a large ASD and was ventilated from birth for respiratory distress syndrome. He died at 12 hours of age despite neonatal ITU care. Post-mortem examination showed highly lobulated kidneys with nodules of blastema and foci of hamartomatous change in the medulla. Prominence of pancreatic islet cells and expansion of hepatic portal tracts were also noted. His mother has minor cervical spine abnormalities. We discuss the differential diagnosis and the difficulty in confidently assigning a diagnosis to this patient, as considerable overlap is becoming evident between Simpson-Golabi-Behmel syndrome and Perlman syndrome.

We report the case of severe hypoxemia attributable to right-to-left shunting through an atrial septal defect after right-sided pneumonectomy that developed in a 70-year-old man. Normal right atrial and pulmonary artery pressures were measured. Right-to-left shunting through a patent foramen ovale is known as a rare complication after pneumonectomy. Our patient, however, demonstrated a true atrial septal defect (septum secundum defect) upon open operative repair of the interatrial connection.

We report the case of a patient, 62-year-old, with a non small cell lung cancer treated by right pneumonectomy followed by chemo and radiotherapy. After surgery appeared a refractory hypoxemia increasing with supine position. Cardiac catheterism showed a right left shunt by reopening of the "foramen ovale". We have performed foramen's occlusion by endovascular method with prothetic material with good result until the death, 14 months later, by neoplasic evolution.

The authors describe an association of atrial septal defect with partial symptoms of the Poland-Moebius syndrome. Both are thought to be caused by developmental disorders of the mesenchyme and ectodermal derivatives. This anomalous association can be accepted as one concept of the subclavian artery blood supply disruption sequence during embryo-genesis.

Atrial septal defects usually give rise to left-to right in the absence of obstruction of the pulmonary outflow tract or pulmonary hypertension. The authors report a case of atrial septal defect with a right-to-left shunt despite normal pulmonary pressures at catheterisation in a 56 year-old-man who had undergone left pneumonectomy 6 months previously. The shunt was responsible for major arterial desaturation aggravated by the left lateral or dorsal decubitus position. Surgical closure of the defect resulted in cure with disappearance of cyanosis and normalisation of blood gases. The physiopathological mechanisms of these right-to-left shunts with normal pulmonary pressures are discussed with reference to previously reported cases in the literature.

Percutaneous mitral balloon valvotomy (PMV) using the Inoue balloon catheter was attempted in 220 consecutive patients with severe symptomatic mitral stenosis. Their age range was 10-63 mean 30 +/- 10 years; 161 patients were females and 59 were males; 29 patients were in atrial fibrillation. Eleven patients were pregnant; 14 patients underwent previous surgical commissurotomy. The procedure was technically successfully performed in 215 (97.7%) patients. The mean fluoroscopy time was 15.5 +/- 6.4 min and mean procedure time was 109 +/- 79 min. Optimal results (group I) was achieved in 207 patients who have mitral score of 7 +/- 1. PMV resulted in decrease in left atrial pressure from 23 +/- 5 to 14 +/- 4 mm Hg (P < 0.001), the mean mitral valve gradient (MVG) decreased from 15 +/- 4 to 6 +/- 3 mm Hg (P < 0.001). The mitral valve area (MVA) by catheter increased from 0.7 +/- 0.2 to 1.7 +/- 0.5 cm2 (P < 0.001) and MVA as determined by echocardiography (2DE) increased from 0.8 +/- 0.1 to 1.9 +/- 0.3 cm2 (P < 0.001). The results were suboptimal in eight patients who have a mitral score of 10 +/- 1 (group II) MVA by catheter increased from 0.7 +/- 0.2 to 1 +/- 0.1 cm2 and Doppler MVA increased from 0.8 +/- 0.1 to 1.3 +/- 0.1 cm2. There were no deaths or thromboembolism. Two patients developed cardiac tamponade; mild mitral regurgitation (MR) developed in 24 patients (11%) and increased by one grade in another 22 patients (10%). Severe MR was encountered in three patients (1.4%). A small ASD (QP/QS < or = 1.3) was detected by oximetry in 5% of patients and by color-flow mapping in 26% of patients. One hundred fifty-eight patients from group I were followed up, for a mean of 32 +/- 12 months; MVA remained at 1.7 +/- 0.4 cm2. Seven patients developed mitral restenosis in group I, and 5 out of 8 patients developed restenosis in group II. We conclude that the hemodynamic results are good and comparable with those reported with double balloon technique. However, the Inoue balloon has several advantages over the double balloon technique: (1) low incidence of mitral regurgitation and ASDs; (2) shorter procedure and fluoroscopy time; and (3) low complication rates and the valve anatomy affects the immediate and late outcome of mitral balloon valvotomy.