Atrial Defect Research Articles

Cardiac Autotransplantation for Removal of Left Atrial Hemangioma and a Review of the Literature

We describe the management of a patient who presented with symptoms of severe congestive heart failure. A 48-year-old man was initially seen in the emergency room, admitted to the hospital, and worked up with a transthoracic echocardiogram, a transesophageal echocardiogram, and a computer tomography scan of the chest. All cardiac valves were normal, as was the left ventricular ejection fraction. A mobile left atrial tumor measuring 6 x 4 x 5 cm was found attached to the left atrial dome, left atrial cuff, and left pulmonary veins. With each systolic atrial contraction, the mass prolapsed into the left ventricle across the mitral valve annulus, inducing a gradient of 19 mm Hg. The workup of the patient was negative for malignancy. The only feasible therapy for this patient was to excise the mass on cardiopulmonary bypass and cardioplegic arrest. At the time of surgery, the findings confirmed that the mass was attached broadly to the left atrial dome wall-epicardium, and the attachments were similar to those of the transesophageal echocardiographic findings. Atrial attachments extended from the base of the heart, along the atrioventricular groove, the left dome of the left atrium, the left atrial cuff, and the anterior aspect of both left pulmonary veins. The tumor could not be adequately excised, and reconstruction of the defect was not feasible with the heart in situ. We therefore decided to explant the heart and excise the tumor with a 0.5-cm margin of healthy tissue. The broad left atrial defect was reconstructed with bovine pericardium. The reconstruction encompassed the dome of the left atrium, the left atrial cuff, and the pulmonary veins. The heart was reimplanted back into the pericardial cavity. The superior vena cava with the retained sinus node was also anastomosed. The pathology diagnosis was a benign cavernous hemangioma. The sinus rhythm recovered following removal of the aortic cross-clamp and reperfusion of the heart. The patient had a rapid recovery and was discharged home on the 12th postoperative day. Placement of a pacemaker was not required because the patient retained the sinus rhythm. A review of the literature on cardiac autotransplantation revealed that this type of surgery has been performed frequently in centers that have a cardiac transplantation program or a surgeon who has cardiac transplantation experience. To our knowledge, this report is the first of cardiac autotransplantation for benign hemangioma.

Percutaneous interventions in adults with congenital heart disease: Expanding indications and opportunities

Percutaneous intervention in adults with congenital heart disease has advanced rapidly in recent years and represents a growing field of invasive cardiology. Valve replacement, stent implantation for native and recurrent coarctation, and ventricular septal defect closure have emerged as attractive alternatives to surgery and have been added to the existing validated procedures (eg, atrial septum defect closure, persistent ductus arteriosus occlusion, and pulmonary valve dilatation). This review summarizes current and expanding indications for transcatheter interventions in adults with congenital heart disease.

Safety and efficacy of ketorolac in children after cardiac surgery

To evaluate the nephrotoxic and opioid-sparing effects of ketorolac in children after cardiac surgery. A retrospective cohort study. A Cardiac Critical Care Unit in a university-affiliated children's hospital. Children less than 18 years of age who underwent low-risk cardiac surgery from July 2002 to December 2005. Among 248 children studied, 108 received ketorolac and 140 did not. The ketorolac group was older, included a larger proportion of atrial septum defect repairs and a smaller proportion of ventricular septum defect repairs compared to the control group. The median change in serum creatinine did not differ between the ketorolac group and the control group (% change [IQR]); 12% [1-25] increase versus 12% [-3 to 31] increase, P = 0.86. On postoperative day 0 or 1, the ketorolac group received less opioids than control group. There was no difference in duration of mechanical ventilation or in length of stay between groups. Ketorolac started in the first 12 h after a low-risk cardiac surgery in children is not associated with a measurable difference in renal function. The data suggest that ketorolac may be effective in reducing the exposure to opioids. Further studies are required to define subsets of children after cardiac surgery who could safely benefit from ketorolac therapy to reduce pain.

Value of intraoperative closure atrial sepetal defect with deficient rims inducted by transoesophageal echocardiography

Objective To evaluate the application valve of intraoperative device closure secundum atrial sepetal defect (ASD) associated with deficient rims inducted by transoesophageal echocardiography (TEE). Methods Twenty-three patients with ASD were diagnosed by transthoracic echocardiography (TTE) ,and underwent an attempted intraoperative device closure through a right antierior minithoraetomy. The positon, type and size and rim of ASD were defined by TEE in preoperation, the placement of the device inducted and evaluated by TEE intraoperation,and followed by TTE after one week. Results Twenty-three patients with ASD were closed excellently. There were not shunt of atrial level in 20 patients, trivial shunt in 3 patients. However,follow-up after one week TTE indicated that all patients had no shunt,and the diameter of right atrium and right ventricle was decrease significantly (P <0.05), and pulmonary artery pressure was less than before (P <0.05). Conclusions It is very important that TEE induct the closure of ASD associated with deficient rims in micro-operative room. Key words: Echocardiography,transesophageal; Heart septal defect,atrial

Evolution of Cardiopulmonary Bypass

At the end of the Second World War, the devastation of many cities in Europe, combined with the problems of reconstruction and loss of financial support, had brought European medical research to a halt. On the other hand, in the United States the war brought about an enormous amount of scientific research through the development of nuclear weapons, the mass production of penicillin, early computers, and a variety of other technological advances, some of which were eventually put to use by medical investigators. In the immediate postwar years, Congress began 3 decades of generous funding for medical research and education through the National Institutes of Health and the Public Health Service. In the 1920s and 1930s, medical research money had been scarce, but after the war there was a flood of money. The National Institutes of Health budget for medical research in 1945 was $180 000. By 1947, it was increased to $4 000 000, in 1950 to $46 000 000, and in 1974 to $1 billion.1 By 2008, the total budget just for the Heart, Lung, and Blood Institute was almost $3 billion. Since colonial times, it had been customary for young American physicians to spend postgraduate time in Vienna, Berlin, London, or Paris. Now the opposite was true. It became important for a young European physician to have worked for a year or so in a US medical center. The world center for medical education and research had shifted from Europe to the United States. Postwar medical progress was also stimulated by several other programs. The Hill-Burton act of 1946 provided funds for community hospital upgrades and construction. Initially this bill provided $75 million per year for 5 years. It was a matching plan in which local hospitals were required to raise two thirds of the new …

Percutaneous closure of complex atrial septum defect guided by real-time 3D transesophageal echocardiography

Sirs, Atrial septal defects of the secundum type represent 7% of all congenital cardiac anomalies. Their morphology varies with regard to the location, size and number [2, 3]. Catheter-based techniques for device closure require detailed anatomic information [6]. In adults transesophageal 2-dimensional (2D) echocardiography generally allows appropriate visualization. For pre-selection of patients with complex defects, however, three dimensional (3D) echocardiography has been reported to provide better qualitative and quantitative information on the dynamic geometry, location and extension as compared with standard 2D echocardiography [4, 7]. Given technical improvement online, 3D reconstruction and guidance of occluder deployment may prove useful, as recently suggested for single defects [5].

Endoscopy-Guided Occlusion of Secundum Atrial Defect Permits Use of Smaller, Cosmetically Superior Thoracotomy

Endoscopy has been introduced into cardiac surgery employing robotic systems. We have developed a new-minimally invasive approach that combines these two technologies for secundum atrial septal defect (ASD). The purpose of this study is to evaluate the efficacy of this approach, paying particular attention to its cosmetic effects. Between November 2004 and April 2006, 22 patients (aged 18 to 62) with ASDs (with sizes 10 to 39 mm) were treated with occlusion apparatus with endoscopy and robotic arm (AESOP 3000, Computer Motion Inc., Santa Barbara, CA, USA) assist. All ASDs were successfully occluded with a mean device size of 26.8 mm (12 to 42 mm). Procedure time was ranged from 30 to 60 minutes. The ICU stay was less than 1 day and hospital stays was 3 to 7 days. No postoperative mortality was noted. During follow-up of 2 to 19 months, no major morbidities occurred. Endoscopic ASD occlusion assisted with robotic is safe and effective with rapid recovery. The cosmetic effect is better results than conventional surgical repair.

Fracture of a GORE HELEX Septal Occluder following PFO closure in a diver

Decompression illness (DCI) is more common in divers with a patent foramen ovale (PFO), and transcatheter PFO closure is being increasingly performed in patients with an episode of DCI who want to continue diving. A range of closure devices are available and the choice in an individual case depends on operator preference and PFO anatomy. The GORE HELEX Septal Occluder, introduced in 1999 primarily for secundum atrial defect closure, is a compliant non self-centering device composed of a wire helical framework on which a microporous membrane is mounted. The device is fixed in place by a unique interlocking mechanism that passes through the center of the device from the left to the right atrial disc, thereby securing it onto the interatrial septum. Here, we present a case of a locking loop fracture and review the literature concerning this unusual complication.

Quantification of left-to-right shunting in adult congenital heart disease: phase-contrast cine MRI compared with invasive oximetry

Atrial septum defects (ASDs), ventricular septum defects (VSDs) and patent ductus arteriosus (PDA) are the most common adult congenital heart defects. The degree of left-to-right shunting as assessed by the ratio of flow in the pulmonary (Qp) and systemic circulation (Qs) is crucial in the management of these conditions. This study compared phase-contrast cine magnetic resonance imaging (PC-MRI), a non-invasive imaging technique, with invasive oximetry for the measurement of shunt volumes during cardiac catheterisation in adults with left-to-right shunting. Both invasive oximetry and shunt quantification by PC-MRI (1.5 T scanner; Sonata, Siemens Medical Solutions) were performed on 21 patients with left-to-right shunting (14 ASD, 5 VSD, 2 PDA) and data on Qp/Qs ratios and left-to-right shunt fraction compared. Mean Qp/Qs ratios assessed by PC-MRI and oximetry were 2.10+/-0.76 and 1.96+/-0.77, respectively (p = 0.37). Mean shunt fraction was 46.3+/-19.6% when calculated by PC-MRI and 42.3+/-20.1% when obtained by oximetry (p = 0.12). There was a strong correlation of Qp/Qs ratios and shunt fraction between both methods (r = 0.61, p < 0.01 and r = 0.84, p < 0.0001, respectively). The two methods had a good agreement according to Bland and Altman plots with a small but non-significant overestimation of Qp/Qs-ratios and shunt fraction by PC-MRI. On receiver operating characteristic analysis, the sensitivity and specificity of PC-MRI to detect an oximetry-derived Qp/Qs ratio of > or =1.5:1 were 93% and 100% at a PC-MRI threshold of a Qp/Qs ratio > or =1.75:1 (area under curve (AUC) = 0.99). Quantification of left-to-right shunting can be performed reliably and accurately by PC-MRI and the data obtained by this method correlate closely to those from invasive oximetry.

Long-term results of surgical repair of ruptured sinus of aortic sinus aneurysm

To summarize the clinic characteristics and effect of surgical repair of ruptured aortic sinus aneurysm. From September 1997 to September 2007, 43 patients with ruptured aortic sinus aneurysm underwent surgical procedures. There were 32 male and 11 female patients. The age ranged from 11 to 50 years old with a mean of (29.0 +/- 11.5) years old. The origins of rupture were the right coronary sinus in 34 patients and the noncoronary sinus in 9 patients. The aneurysms ruptured into the right ventricle in 30 patients, the right atrium in 8 patients, the right ventricle and right atrium in 3 patients, and the ventricular septum and then the right ventricle in 2 patients. Associated cardiac anomalies included ventricular septal defect in 26 patients, aortic regurgitation in 15 patients, infectious endocarditis in 8 patients, tricuspid regurgitation in 6 patients, atrial septum defect in 4 patients, mitral valve regurgitation in 2 patients, patent ductus arteriosus in 2 patients, and pulmonary valve vegetation in 1 patient. All the patients underwent the repair of ruptured aortic sinus aneurysm and correction of associated anomalies with cardiopulmonary bypass. There were no deaths after the operation and during the follow-up. The complications, including acute heart failure and III atrioventricular block, occurred in 5 patients. Follow-up was 6 to 120 months with a mean of (68.0 +/- 17.7) months. Two patients underwent reoperation for aortic valve replacement at the 6(th) and 8(th) year after the first operation. There were 2 patients which the aortic regurgitation deteriorated from grade I to II. Repair of ruptured aortic sinus aneurysm presents a satisfactory result. Aggressive treatment in early time, prevention of post-operative complications and long-term follow-up are recommended in the treatment for patients of ruptured aortic sinus aneurysm with infectious endocarditis and aortic regurgitation.

Prevalence of Pericardial Effusions in Children With Large Atrial or Ventricular Septal Defect

Recently, a high percentage of adults with isolated secundum atrial septal defects (ASD) and ventricular septal defects (VSD) undergoing surgical repair had a circumferential pericardial effusion (PE), preoperatively. It was our impression that PEs are uncommon in children with unrepaired ASD or VSD. To evaluate this impression, we performed a retrospective study to determine the prevalence of PE in children with ASD compared with those with VSD and with children with normal cardiac anatomy. The preoperative echocardiograms of 90 patients with isolated surgical grade secundum ASDs, 75 patients with isolated VSDs, and 91 normal patients were reviewed to evaluate for the presence or absence of pericardial effusions and also the septal defect size. Only 1 patient (ASD group) of the total 256 had a global PE (0.4%). No large effusions were found in any group. Eighty-one children (32%) had any effusion present in the pericardial space. The prevalence of these small PEs was not significantly different between the ASD (36/90, 40%) and VSD (23/75, 31%, p=0.25) groups. The patients with ASD, however, had a significantly higher prevalence of PEs compared with the control group (22/91, 24%, p<0.03). In conclusion, the presence of global PEs in children with surgical grade ASD or patients with VSD is rare, unlike that reported in adults with the same lesions. Trace effusions are found more commonly in children with ASD than those in VSD or in normal children, which may herald a potential for larger effusions with a longer time before defect closure.

Atrial and ventricular lead insulation defects with resulting inappropriate shocks and end-of-life of the ICD pulse generator in a young bodybuilder with congenital long-QT-syndrome

We report on a 19-year-old male with a congenital Long-QT syndrome who was admitted to our hospital because of insulation defects of both atrial and ventricular ICD leads resulting in inappropriate delivered shocks and a non-responding pulse generator during telemetrical evaluation. The insulation defects led to multiple arc marks within the ICD pocket and there was a short circuit between denuded leads and the electrically active pulse generator. The high current flow generated sufficient heat to damage several circuits of the generator. A new ICD system was implanted successfully and the postoperative course has been remained uneventful for almost 6 years.

Baffle puncture guided by transoesophageal echocardiography in a patient with dextrocardia and Mustard correction

A baffle puncture is a challenging procedure but can be safely done using direct visualization of the region of interest. To our knowledge, however, it has never been performed in a patient with dextrocardia. We present a 62-year-old male with dextrocardia, right isomerism, congenitally corrected transposition of the great arteries, persistent left-sided superior and inferior caval veins, atrial septum defect, and pulmonary valve stenosis. The atrial septum defect was surgically closed with a Teflon patch, a variant Mustard operation was performed, and also a prosthetic tricuspid valve was implanted. The patient developed multiple episodes of atrial tachycardia leading to acute heart failure on many occasions. An electrophysiological study was undertaken in order to create a bi-atrial electro-anatomical map. Owing to the presence of a prosthetic tricuspid valve, the femoral venous access was used and a baffle puncture was performed using continuous monitoring with fluoroscopy and transoesophageal echocardiography (TEE). The baffle puncture was successful and the tachycardia was ablated in the systemic venous atrium. To our knowledge, we present the very first case report demonstrating a successful baffle puncture in a patient with dextrocardia and Mustard correction. Direct imaging using TEE seems to be a very useful tool for guiding the puncture.

Interventional cardiology in Switzerland 2006

Background: Since 1987, a nationwide survey of percutaneous cardiac interventions is performed annually in Switzerland, which allows recognition of contemporary trends and comparison with other countries. Methods: Volume and type of procedures performed during the year 2006 were collected by means of a standardised questionnaire from all adult percutaneous cardiac intervention centres in Switzerland. Results: During the year 2006, 36817 coronary angiographies (CA; 2005: 36436; +1.0%) and 17061 percutaneous coronary interventions (PCI; 2005: 16624; +2.6%) were performed in 27 centres (5 university hospitals, 9 public, nonuniversity hospitals, and 13 private hospitals) by 193 operators (64 of them performing only diagnostic studies). Ninety-three percent of PCI procedures were performed ad hoc, and 78% of them were single-vessel interventions. Stents were used in 89% of all PCI (2005: 91%) procedures with drug-eluting stents (DES) being responsible for the largest share (82% of all stents) (2005: 78%). Emergency procedures (primary PCI or rescue PCI after failed thrombolysis) accounted for 20% of interventions (2005: 19%). Glycoprotein IIb/IIIa inhibitors were used in 19% of PCI (2005: 23%) procedures. In addition, valvuloplasties of mitral, aortic, and pulmonary valves were performed in 42, 21, and 5 cases, respectively. Finally, percutaneous closure of a patent foramen ovale and an atrial deptal defect were performed in 515 (2005: 510) and in 88 (2005: 94) cases, respectively. Conclusions: The increase in the number of CA and PCI has flattened during 2006. The use of DES remained high, though the previously rapidly growing utilisation of these devices reached a plateau. The number of procedures for the percutaneous closure of patent foramen ovale and atrial septal defect has stabilised.

Ablation of Atrial Tachycardia From a Giant Left Superior Vena Cava Using Integration With Computed Tomographic Imaging

A 49-year-old woman affected by the Turner syndrome with a history of repaired congenital cardiomyopathy and persistent atrial tachycardia was referred to our center for radiofrequency ablation. At 11 years of age, she had undergone a surgical closure of an ostium secundum atrial septal defect (direct suture) combined with ligation of a persistent left superior vena cava, the embryological precursor of the ligament of Marshall, at the level of its connection to the coronary sinus. At hospitalization, her electrocardiogram showed an atrial tachycardia (cycle length 375 ms) with long phases of 1:1 atrioventricular conduction. Before the procedure, a computed tomography (CT) scan was performed to merge the anatomic data with the electrophysiological findings during the procedure (CARTO-MERGE, Biosense Webster, Inc, Diamond Bar, Calif). The preacquired CT image, processed with CARTO-MERGE system, showed the presence of a giant left superior vena cava (isolated from the coronary sinus) and a sinus venosus defect (SVD) associated with a right-sided anomalous pulmonary venous connection. In detail, both right superior pulmonary vein and right inferior pulmonary vein drained into the superior …

To the Editor:

Introduction: Transseptal puncture (TSP) is the conventional approach to assess the left atrial chamber. This technique has been widely used in interventional cardiology and, in the last years, this approach is mostly applied to electrophysiologic procedures. For atrial fibrillation (AF) ablation, two or more transseptal sheaths are often positioned in the left atrium in the majority of centers, therefore requiring two or more transseptal punctures. Theoretically, double puncture could bear additional risks or could increase the risk of persistence of septal defects. We reported the results of a retrospective analysis of a single transseptal puncture as a simplified approach for positioning multiple catheters in the left atrium during AF ablation. Methods: Between November 2003 and November 2005, 1,150 consecutive patients (mean age 54 ± 8 years, 75% men) were considered for AF ablation and underwent single transseptal puncture for positioning multiple catheters in the left atrium. Results: In only 6 of 1,150 (0.5%) cases a second transseptal puncture was required to position the ablation catheter in the left atrium. Neither acute complications related to transseptal catheterization such as atrial or aortic perforations, pericardial tamponade, thrombotic formation, or air embolism, nor complications due to the attempts of crossing the septum with the ablation catheter were reported. In all cases, each mapping catheter was properly maneuvered at different sites in the left atrium and at the pulmonary veins. In no patient was a residual septal atrial defect after the transseptal maneuvers detected during a mean follow up of 14 ± 2 months. Conclusions: This retrospective study on a large cohort of patients shows that single transseptal puncture for positioning two or more catheters in the left atrium for AF ablation is a highly successful and safe maneuver with a very low morbidity in the majority of patients. This may avoid potential complications related to a second transseptal puncture.

Bilateral pulmonary artery banding for hypoplastic left heart syndrome and related anomalies

Bilateral pulmonary artery banding is considered an option for initial palliation in high-risk patients with hypoplastic left heart syndrome or related anomalies. However, there are potential interim morbidities, including ductal constriction, compromised growth of the ascending aorta, and pulmonary artery stenosis at the banding site. In addition, there are still controversies regarding the optimal timing and choice of the second-stage operation. Between February 2003 and October 2005, twelve high-risk patients with hypoplastic left heart syndrome or related anomalies underwent bilateral pulmonary artery banding. An atrial septal defect was created simultaneously when the septum was intact or the defect was restrictive. After the procedure, intravenous prostaglandin E(1) was continuously administered. The median weight was 3.0 kg (range 1.8-3.6 kg), and the median age was 16 days (range 0-27 days). Atrial septum defect creation was performed simultaneously in two patients. Ten of the twelve patients were discharged from the hospital. One patient died of progressive multiple organ failure and another patient died of cerebral hemorrhage. Eight patients developed various degrees of ductal constriction and required dose adjustment of prostaglandin E(1). Two patients underwent ductal arteriosus stent placement: one was unsuccessful owing to the size mismatch between the stent and the ductus. The diameter of the ascending aorta remained unchanged until the time of the second-stage operation, except in two patients who had a sufficiently sized aorta at the time of pulmonary artery banding. Pulmonary arterial branch stenosis developed at the banding site in three patients. At the time of the second-stage operation, the Norwood procedure was performed in seven patients, biventricular repair in two, and Norwood plus Glenn procedure in one. Bilateral pulmonary artery banding is an option to salvage high-risk neonates with hypoplastic left heart syndrome or related anomalies. However, it is associated with high interstage morbidity, and patients may benefit from early conversion to the Norwood operation.

Single Centre Experience with GORE-HELEX Septal Occluder for Closure of PFO

Several devices are available for percutaneous patent foramen ovale (PFO) closure. Over the past 3 years our planned treatment strategy of PFO closure has been routine use of the GORE-HELEX septal occluder device, followed by 6 months of aspirin therapy. We present the safety and feasibility of routinely using this device for all patients undergoing percutaneous PFO closure, with 3-month transthoracic echocardiogram (TTE) follow up. In total, 75 adult patients (44.0+/-11.7 years; 45.3% male) were referred for PFO closure during the study period. All patients underwent echocardiography prior to PFO closure. In 5 patients no PFO was found, and 1 patient had an atrial secundum defect closed using the Amplatzer septal occluder. Of the 69 remaining patients with PFO, 68/69 (98.6%) underwent closure with the GORE-HELEX device. Six of 69 cases required device retrieval and 5 of 6 were replaced successfully with a second GORE-HELEX device. One of the 6, a large PFO associated with atrial septal aneurysm, was replaced using the Amplatzer septal occluder. There were no major complications. At 3-month follow up, 65/68 (95.6%) had no residual shunt on TTE, and 3 patients had small residual shunts thought to be related to incomplete endothelialisation at 3 months. In conclusion, percutaneous PFO closure using the GORE-HELEX septal occluder device is safe and feasible, with no major peri-procedural complications and excellent short-term results at 3-month follow up.

An unusual diagnosis of atrial shunt defect by magnetic resonance imaging

Paroxysmal atrial arrhythmias especially atrial fibrillation are frequently encountered in adult patients with atrial septal defect. However, the diagnosis of atrial defect can be difficult. Thransthoracic echocardiography, the mostly utilized cardiac technique, has shown a limited ability to identify small atrial defects. Transesophageal echocardiography has shown high accuracy to identify but it isn't well tolerated of the patients. Recently, the utility of multislice computed tomography in the evaluation of direction, location, and size of shunt flow in congenital heart disease has been demonstrated. Cardiac magnetic resonance imaging (MRI) is a recent imaging technique that permits with high spatial resolution and without ionising radiation an accurate identification of many cardiovascular diseases. We report an unusual detection of an atrial defect by phase-contrast cine MRI in a patient clinically suspected of arrhitmogenic right ventricular displasia.

A complex clinical case linking genetic disorders and infectious disease

Dr. Leopoldo Di Iorio: On the basis of this clinical presentation, four main hypotheses were considered and simultaneously investigated. First, a cardiogenic shock was considered (subjects with Down’s syndrome can often have multiple cardiac abnormalities, such as atrium-ventricular channel, ventricular or atrial defects, Fallot’s tetralogy), but echocardiography excluded significant alterations of the parietal kinesis or structural abnormalities. Second, the presence of genital mycosis, marked hypocalcaemia, several symptoms indicative of acute adrenocortical deficiency (ions disorders, hypoglycaemia, hypotension, bradycardia) were all suggestive of type I polyglandular autoimmune syndrome (PAS, a syndrome characterised by muco-cutaneous candidiasis, hypoparathyroidism and Addison’s disease) [1]. However, results from specific laboratory tests (presence of hypercortisolaemia associated with normal levels of ACTH and PTH) definitely ruled out this clinical hypothesis. Third, we excluded a neurogenic shock by performing a brain CT, which did not document signs of ischaemic or haemorrhagic lesions, but found an enlargement of the IV ventricle associated with congenital olivary cerebello-pontine atrophy.