Congenital Atrial Septal Defect Research Articles

Lutembacher’s Syndrome with Small Atrial Septal Defect Diagnosed by Transthoracic and Transesophageal Echocardiography that Underwent Mitral Valve Replacement

Lutembacher's syndrome is a rare clinical combination of congenital ostium secundum atrial septal defect associated with acquired mitral valve stenosis (usually rheumatic). This unusual cardiac entity is difficult to diagnose clinically because each lesion alters the hemodynamics and clinical characteristics of the other. The resulting clinical manifestations depend chiefly on the size of the defect, the severity of the mitral stenosis, and the compliance of the right ventricle. We present a classic case of Lutembacher's syndrome and illustrate the pitfalls and advantages of echocardiography in the correct diagnosis of the syndrome.

Commissurotomie mitrale percutanée et syndrome de Lutembacher

Introduction. – Lutembacher syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. This condition is usually treated surgically by mitral valve operation with concomitant closure of the atrial septal defect. Materials and methods. – Between 1993 and 2003, 4 patients with congenital Lutembacher syndrome had percutaneous mitral commissurotomy without closure of the atrial septal defect at our institution. The 4 patients were very symptomatic with right-sided heart failure signs and NYHA functional class III-IV. Results. – The procedure was carried out successfully for the four patients. Mitral valve area increased from 0,87 to 1,97 cm 2 at mean; left atrial pressure decreased from 28,2 to 12,7 mmHg and the mean valve mitral gradient was reduced from 15,5 to 3,9 mmHg. Functional and clinical improvement was observed in all the cases. During a mean follow up of 55 ± 29 months, our 4 patients remain pauci symptomatic under medical treatment. Conclusion. – The percutaneous treatment of the Lutembacher syndrome is currently a possible alternative to the surgery among patients having an anatomy favourable to the procedure.

Echocardiographic assessment of left and right heart hemodynamics in a patient with lutembacher's syndrome

We present a case of a 53-year-old woman with intractable shortness of breath that was originally ascribed to bronchiolitis obliterans organizing pneumonia. Subsequently evaluation by echocardiography and cardiac catheterization revealed that she had Lutembacher's syndrome, an uncommon combination of congenital atrial septal defect (ASD) and acquired mitral stenosis that is difficult to diagnose clinically. Our case illustrates the pitfalls and advantages of echocardiographic assessment of the mitral valve area (MVA) and the left atrial pressure (LAP). The pressure half-time method used most commonly for estimating MVA echocardiographically is inaccurate and may lead to underestimation of the severity of mitral stenosis in patients with Lutembacher's syndrome. On the other hand, the presence of ASD provides an additional method of calculating LAP, the most important determinant of symptoms in patients with mitral stenosis.

Development of heart failure and congenital septal defects in mice lacking endothelial nitric oxide synthase.

Nitric oxide (NO) produced by endothelial NO synthase (eNOS) plays an important role in the regulation of cell growth, apoptosis, and tissue perfusion. Recent studies showed that mice deficient in eNOS developed abnormal aortic bicuspid valves. The aim of the present study was to additionally investigate the role of eNOS in heart development. We examined postnatal mortality, cardiac function, and septum defects in eNOS(-/-), eNOS(+/-), and wild-type mice. Postnatal mortality was significantly increased in eNOS(-/-) (85.1%) and eNOS(+/-) (38.3%) compared with wild-type mice (13.3%, P<0.001). Postmortem examination found severe pulmonary congestion with focal alveolar edema in mice deficient in eNOS. Heart shortening determined by ultrasound crystals was significantly decreased in eNOS(-/-) compared with wild-type mice (P<0.05). Congenital atrial and ventricular septal defects were found in neonatal hearts. The incidence of atrial or ventricular septal defects was significantly increased in eNOS(-/-) (75%) and eNOS(+/-) (32.4%) neonates compared with those of the wild-type mice (4.9%). At embryonic days 12.5 and 15.5, cardiomyocyte apoptosis and myocardial caspase-3 activity were increased in the myocardium of eNOS(-/-) compared with wild-type embryos (P<0.01), and increases in apoptosis persisted to neonatal stage in eNOS(-/-) mice. Deficiency in eNOS results in heart failure and congenital septal defects during cardiac development, which is associated with increases in cardiomyocyte apoptosis. Our data demonstrate that eNOS plays an important role in normal heart development.

Changes of the Children Myocardium Lipid Composition under Surgical Intervention by the Use Cold Crystalloid Cardioplegia

Changes of the children's myocardium lipid composition with congenital atrial septal defect (ASD) during a surgical intervention with the use of cold crystalloid cardioplegia were shown for the first time. At ischemia, which developed during cardioplegia, the quantities of phosphatidylinositol, phosphatidylcholine and diphosphatidylglicerol were enhanced. It was found that the quantity of short-chain fatty acids decreased almost two-fold and the content of some long-chain fatty acids esterified in phospholipids was raised. The quantity of w6-fatty acids (eicosatrienoic, arachidonic) was increased in cholesterol esters fraction. The significant accumulation of free miristoleinic, linoleic and some other free fatty acids was shown under cardioplegia. The total amount of cholesterol esters decreased by three times and the ratio of the phospholipids/cholesterol esters increased. Reperfusion caused additional changes of myocardium lipid composition. The amount of lysophosphatidylethanolamine, w6 free fatty acids (including arachidonic acid) enhanced. At the first minutes of the reperfusion, the amount of short-chain and some long-chain fatty acids esterified into phospholipids and cholesterol esters returned to initial level. We have shown that the surgery of the ASD closure at the children heart caused considerable changes of myocardium lipid composition. The amount of free arachidonic acid under reperfusion increased nearly four times.

Long-term outcomes excellent for atrial septal defect repair in adults.

Congenital atrial septal defect repair is safe and effective in patients of almost any age. Long-term survival among adults is excellent, although children generally appear to fare even better. Our 25-year study of outcomes among adults who underwent suture or patch closure found that survival exceeded 90%. We discuss our observations on use of transesophageal echocardiography, indications for cardiac catheterization, and continuing questions about atrial septal defect in adults.

Protective effect of Salvia Miltiorrhizae Composita on myocardium in patients undergoing open heart surgery

To observe the protective effect of Salvia Miltiorrhizae Composita (SMC) on myocardium injuries induced by oxygen free radicals from ischemic reperfusion in patients undergoing open heart surgery. Eighteen patients with either congenital ventricular septal defect (VSD) or atrial septal defect (ASD) were divided randomly into control group and treated group, 9 in each. Patients in the treated group were administered intravenously SMC 200 mg/kg before operation and in rewarming period, while patients of the control group received the same volume of balanced salt solution. Serum malonyldialdehyde (MDA), creatine phosphokinase (CPK) and lactate dehydrogenase (LDH) were measured before operation, 30 min after myocardium ischemia, 30 min after reperfusion, 30 min after cardiopulmonary bypass and 24 hours after operation. Serum MDA, CPK and LDH levels in the treated group at both myocardium ischemic period and ischemic reperfusion period were significantly higher than those at before operation (P < 0.05, P < 0.01). In the treated group, levels of the 3 indexes at myocardium ischemic period increased slightly in comparison with those before operation (P > 0.05), and the levels of them at ischemic reperfusion period were significantly higher than those before operation (P < 0.05), but there was no significant difference between the levels at ischemic reperfusion period and those at 30 min after myocardium ischemia. Comparison between the two groups showed that the serum MDA, CPK and LDH levels were all significantly higher in the control group than those in the control group respectively in all periods (P < 0.01). SMC could effectively protect myocardium from ischemia and ischemic reperfusion by attenuating the free radical damage and inhibiting lipid peroxidation.

成人型心房中隔欠損症における心房性不整脈に関する検討 心房細動に及ぼす加齢の影響

Clinical characteristics of aging in congenital atrial septal defect (ASD) without significant right-to-left shunt (> 10%), were examined especially focusing on atrial arrhythmias such as atrial fibrillation (Af) and atrial premature contraction (PAC) in serial 48 patients, aged 42.6 +/- 2.3, ranging 16-67 years, Af was found in 12 (25%) out of 48 patients. Comparing patients with Af and those without Af, the average age (52.8 +/- 2.6 vs 38.6 +/- 2.8 years, p < 0.05), functional class of New York Heart Association (2.0 +/- 0.2 vs 1.6 +/- 0.1, p < 0.05) and left atrial dimension (41.4 +/- 1.1 vs 34.7 +/- 1.2 mm, p < 0.01) were significantly higher in the patients with Af than those without Af. Hemodynamic variables, i.e., mean right or left atrial pressure, Pp/Ps, Qp/Qs, left-to-right shunt, electrocardiographic R/S ratio in the lead V1 and tricuspid regurgitation had no relation either with aging or Af. Furthermore, daily PAC counts on Holter electrocardiograms significantly correlated with the age of the patients (r = 0.642, p < 0.05). These results suggest that the incidence of Af in congenital ASD is significantly related with aging/duration of ASD, but not with hemodynamic variables.

Transesophageal echocardiography with color Doppler during interventional catheterization.

Echocardiography has been used during interventional cardiac catheterization for guidance of the procedure and assessment of results. However, limited echocardiographic windows and logistical difficulties make conventional echocardiography during interventional catheterization impractical and of limited value. Transesophageal echocardiography (TEE) with Doppler color flow mapping is more useful than conventional echocardiography for guidance of interventional catheterizations. Device closure of congenital atrial and ventricular septal defects (ASDs and VSDs) and of postoperative residual interatrial and interventricular communications, balloon valvuloplasty, and Brockenbrough atrial septal puncture with ASD creation have been performed under combined fluoroscopic and TEE guidance. Transesophageal echocardiography can be performed continuously throughout the procedure, allowing simultaneous fluoroscopic and echocardiographic assessment of catheter position. Localization of individual device arms during transcatheter device closure of ASDs and VSDs is easier and more accurate when TEE is used, resulting in a lower incidence of incorrect device positioning. Doppler color flow mapping is used to determine number, location, and size of defects, to detect residual shunts after device closure, and to assess valve insufficiency after balloon valvuloplasty and other procedures. Combined TEE and fluoroscopic guidance of interventional procedures, with echocardiographic assessment of results, reduces the amount of radiation and contrast used during the procedure, allowing performance of additional interventional procedures during the same catheterization. Transesophageal echocardiography is indicated during interventional procedures in which simultaneous Doppler color flow mapping and precise localization of catheter, balloon and/or device position will result in a higher success rate and decreased morbidity.

The value of left parasternal impulse recordings in the assessment of mitral regurgitation.

Left parasternal cardiograms were recorded at the fifth intercostal space in 30 patients with mitral regurgitation. Early systolic outward movements (E), late systolic outward movements (LOM), and the ratio of the LOM area to the total area occupied by the left parasternal cardiogram (LPC area) were examined. Thirteen patients had pure mitral regurgitation due to ruptured chordae tendineae (ten patients) or "floppy mitral valve" (three patients). In these patients LOM/E correlated significantly with the regurgitation volume determined by the difference between angiographic and Fick stroke volumes, ( r = 0.93, P &lt; 0.01), and with the height of the "v" wave in the pulmonary artery wedge pressure tracings ( r = 0.79; P &lt; 0.01). Simultaneous recordings of left parasternal movements and pulmonary wedge pressures in eight patients in this group showed a close relationship between LOM and "v" waves. Fourteen patients had rheumatic heart disease and mitral regurgitation associated with mitral stenosis of varying degrees of severity. In this group there was no significant correlation between LOM/E or LOM area/LPC area and the regurgitation volume or the height of the "v" wave. Of three patients who had congenital atrial septal defect in addition to mitral regurgitation, none had prominent LOM in the left parasternal cardiogram. This study suggests that if mitral stenosis and atrial septal defect can be excluded on clinical evidence, the left parasternal cardiogram may be used to obtain a noninvasive assessment of the severity of mitral regurgitation, particularly in patients with recent onset of the disease.

Relationship between the ECG, ventricular activation, and the ventricular conduction system in ostium primum ASD.

A combined electrophysiologic-anatomic study was performed in a dog with a congenital atrial septal defect (ASD) primum. Comparisons were made between the dog and electrophysiologic data obtained in four patients with ASD primum. The ECGs and VCGs in both the dog and patients revealed the characteristic features of superior orientation, counterclockwise rotation, and slow-rightward terminal forces in the frontal plane QRS. In both the dog and the patients, epicardial ventricular activation revealed early posterior LV wall excitation and later anterior left ventricular (LV) depolarization. Also, there was a marked delay in the activation of the RV in both dog and patients. In the dog, Purkinje (Pj) potentials were recorded from the endocardium of the anterior and posterior LV wall and the anterior RV wall. The endocardial-Pj activation times of these three regions were correlated with the measured lengths of the three major divisions of the conduction system supplying them. Premature activation of the posterior LV Pj and delayed activation of the anterior LV and RV Pj was observed. The marked asynchrony of Pj activation was due to a developmental asymmetry of the ventricular conduction system, characterized by markedly different lengths of the three major divisions. Purkinje conduction velocity was 1.5 m/sec, and there were no areas of block or slow conduction. The agreement between the dog and human data indicated an identical mechanism for patients with ASD primum. The terms left anterior-superior hemiblock and right bundle branch block are inappropriate descriptions of this lesion.

Traumatic rupture of the right papillary muscle in a patient with congenital atrial septal defect

Traumatic rupture of two right papillary muscles in a man with a congenital atrial septal defect resulted in a reversal of flow through the defect, with the sudden appearance of central cyanosis, and caused severe circulatory failure of a predominantly “forward” type. The patient survived for 7 weeks after the injury and died from thromboembolic complications shortly after cardiac catheterization.

CONSTRICTIVE PERICARDITIS COMPLICATING DISSEMINATED LUPUS ERYTHEMATOSUS.

A case of constrictive pericarditis complicating disseminated lupus erythematosus is reported in a 58-year-old man. Hemodynamic studies documented the diagnosis and also confirmed the clinical suspicion of coexistent atrial septal defect. Measurement of left ventricular volume by a thermodilution technic augmented information available from routine studies. Pericardiectomy and closure of the atrial septal defect relieved the mechanical lesion. The case is also unique in its documentation of the coexistence of three different forms of heart disease—congenital atrial septal defect, pericardial constriction from lupus erythematosus, and rheumatic valvular disease.

The spetial vectorcardiogram in proved congenital atrial septal defect

The sVCG was recorded with the equilateral tetrahedral reference system in 20 patients with proved atrial septal defects (8 primum and 12 secundum defects). The QRSsÊ loops of the patients with primum defects were uniform in configuration, direction of rotation, and orientation. This was also true for the patients with secundum defects. The QRSsÊ loops of the patients with primum defects were oriented to the left, superiorly and posteriorly, and rotated counterclockwise, whereas those of the patients with secundum defects were oriented essentially 180 degrees away, that is, to the right, inferiorly and anteriorly, and rotated clockwise. Because of the consistency in the findings, and because the differences between the two groups were so readily recognizable, even to the untrained person, the sVCG could be useful in the diagnosis of atrial septal defects.