Comparison of m-mode echocardiographic left ventricular mass measured using digital and strip chart readings: The Atherosclerosis Risk in Communities (ARIC) study

In contrast with transthoracic echocardiography, transesophageal echocardiography provides a sure way to make the diagnosis of sinus venosus atrial septal defect; on the other hand this abnormality is more complex than that seen with the secundum atrial septal defect, and inexperienced operators may fail to recognize properly the defect. In front of a high reported sensitivity using transesophageal echocardiography, specificity is difficult to assess, due to possible underreporting of diagnostic errors. We describe a false positive diagnosis of sinus venosus atrial septal defect, in the setting of enlarged right chambers of the heart because of pressure overload. Modified anatomy of the heart, together with the presence of a prominent linear structure(probably Eustachian Valve) and an incomplete examination in this case made image interpretation very prone to misinterpretation. In this anatomical setting transesophageal longitudinal "bicaval" view may be sub-optimal for examining the atrial septum, potentially showing false images that need to be known for correct image interpretation. Nonetheless, a scan plane taken more accurately at the superior level would have demonstrated/excluded the pathognomonic feature of sinus venosus atrial septal defect in the high atrial septum, between the fatty limbus and the inferior aspect of the right pulmonary artery; moreover TEE allows morphological information about the posterior structures of the heart that need to be investigated in detail for a complete diagnosis.     

Atrial septostomy benefits severe pulmonary hypertension patients by increase of left ventricular preload reserve

At present, it is unknown why patients suffering from severe pulmonary hypertension (PH) benefit from atrial septostomy (AS). Suggested mechanisms include enhanced filling of the left ventricle, reduction of right ventricular preload, increased oxygen availability in the peripheral tissue, or a combination. A multiscale computational model of the cardiovascular system was used to assess the effects of AS in PH. Our model simulates beat-to-beat dynamics of the four cardiac chambers with valves and the systemic and pulmonary circulations, including an atrial septal defect (ASD). Oxygen saturation was computed for each model compartment. The acute effect of AS on systemic flow and oxygen delivery in PH was assessed by a series of simulations with combinations of different ASD diameters, pulmonary flows, and degrees of PH. In addition, blood pressures at rest and during exercise were compared between circulations with PH before and after AS. If PH did not result in a right atrial pressure exceeding the left one, AS caused a left-to-right shunt flow that resulted in decreased oxygenation and a further increase of right ventricular pump load. Only in the case of severe PH a right-to-left shunt flow occurred during exercise, which improved left ventricular preload reserve and maintained blood pressure but did not improve oxygenation. AS only improves symptoms of right heart failure in patients with severe PH if net right-to-left shunt flow occurs during exercise. This flow enhances left ventricular filling, allows blood pressure maintenance, but does not increase oxygen availability in the peripheral tissue.     

An infant with diaphragmatic hernia, anophthalmia and cardiac defect: evaluation by magnetic resonance imaging autopsy

We present an infant with diaphragmatic hernia, anophthalmia and cardiac defect evaluated by magnetic resonance imaging (MRI) autopsy. This female infant was born at 39th weeks by vaginal delivery and presented with diaphragmatic hernia, anophthalmia, cardiac defect and died due to respiratory problems at 28th hours of life. MRI autopsy showed internal organ abnormalities including congenital hernia of the left diaphragm, secondary hypoplasia of the left lung, atrial and ventricular septal defect, dilatation of calices of the kidneys, bilateral anophthalmia, hypoplasia of the optic nerves, hyperintensity of pituitary gland possibly due to bleeding and a cyst of the septum pellucidum. This article shows that MRI autopsy is a valuable method for the evaluation of cases with congenital anomalies if autopsy is not possible.     

CARDIAC CATHETERIZATION IN ADULT CONGENITAL HEART DISEASE—A Preliminary Report

The diagnosis of uncomplicated ventricular septal defect and uncomplicated patent ductus arteriosus presents few hazards. Differential diagnosis of atrial septal defect and of transposed pulmonary veins is technically more difficult. The complex nature of most types of cyanotic congenital heart disease requires the combined use of catheterization, determination of circulation times, ventilation studies, and possibly angiocardiography.Reports of eight cases in which cardiac catheterization was carried out illustrate the common forms of acyanotic and cyanotic heart disease and the factors in diagnosis.     

Cardiac catheterization in adult congenital heart disease; a preliminary report

The diagnosis of uncomplicated ventricular septal defect and uncomplicated patent ductus arteriosus presents few hazards. Differential diagnosis of atrial septal defect and of transposed pulmonary veins is technically more difficult. The complex nature of most types of cyanotic congenital heart disease requires the combined use of catheterization, determination of circulation times, ventilation studies, and possibly angiocardiography. Reports of eight cases in which cardiac catheterization was carried out illustrate the common forms of acyanotic and cyanotic heart disease and the factors in diagnosis.     

1H nuclear magnetic resonance studies of sarcoplasmic oxygenation in the red cell-perfused rat heart.

The proximal histidine N delta H proton of deoxymyoglobin experiences a large hyperfine shift resulting in its 1H nuclear magnetic resonance (NMR) signal appearing at approximately 76 ppm (at 35 degrees C), downfield of the diamagnetic spectral region. 1H NMR of this proton is used to monitor sarcoplasmic oxygen pressure in isolated perfused rat heart. This method monitors intracellular oxygenation in the whole heart and does not reflect oxygenation in a limited region. The deoxymyoglobin resonance intensity is reduced upon conversion of myoglobin to the ferric form by sodium nitrite. 1H resonances of the N delta H protons of the alpha and beta subunits of bovine deoxyhemoglobin do not interfere with the measurement of myoglobin deoxygenation in blood-perfused rat heart. We find that steady-state myoglobin deoxygenation is increased progressively (and reversibly) as oxygenation of the perfusing medium is decreased in both saline and red blood cell-perfused hearts at constant work output. An eightfold increase in the heart rate of the blood-perfused heart resulted in no change in the deoxymyoglobin signal intensity. Intracellular PO2 of myoglobin-containing cells is maintained remarkably constant in changing work states.     

Noninvasive cineangiography by magnetic resonance global coherent free precession

Cardiovascular disease is primarily diagnosed using invasive X-ray cineangiography. Here we introduce a new concept in magnetic resonance imaging (MRI) that, for the first time, produces similar images noninvasively and without a contrast agent. Protons in moving blood are 'tagged' every few milliseconds as they travel through an arbitrary region in space. Simultaneous with ongoing tagging of new blood, previously tagged blood is maintained in a state of global coherent free precession (GCFP), which allows acquisition of consecutive movie frames as the heart pushes blood through the vascular bed. Body tissue surrounding the moving blood is never excited and therefore remains invisible. In 18 subjects, pulsating blood could be seen flowing through three-dimensional (3D) space for distances of up to 16 cm outside the stationary excitation region. These data underscore that our approach noninvasively characterizes both anatomy and blood flow in a manner directly analogous to invasive procedures.     

Transcatheter leadless permanent pacemaker in complex congenital heart disease with interrupted inferior vena cava: A challenging implantation

31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Transcatheter leadless pacemaker was deployed in her RV septum despite some unforeseen technical problems. This patient had intrahepatic interruption of IVC with Azygous continuation draining into SVC but this altered venovascular course was detected only fluoroscopically midway during the pacemaker implantation procedure and this was not detected in the preprocedural transthoracic echocardiography. This abnormal venous course was clearly demonstrated in the cardiac CT which was performed only after completion of the pacemaker implantation procedure in this patient. The technical challenges encountered mainly were mostly during the manipulation of the 27F delivery catheter of Micra through this altered cardiovascular anatomy via transfemoral approach and also due to the presence of septal defects. Thus, transcatheter leadless permanent pacemaker was implanted successfully through transfemoral access in this complex congenital heart disease with interrupted IVC and azygous continuation. Besides transthoracic echocardiography, it may be better to perform transesophageal echocardiography or even preferably radiological imaging like cardiac CT or MRI prior to transcatheter leadless pacemaker implantation in patients with complex congenital heart disease to understand the cardiovascular anatomy and plan the procedure.     

A case of macrocephaly-cutis marmorata telangiectatica congenita and review of neuroradiologic features

Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is characterized by macrocephaly, cutis marmorata, capillary malformations, toe syndactily, joint laxity and pre-natal overgrowth. Cerebral abnormalities might also be seen. We reported cerebral magnetic resonance imaging (MRI) findings of a case with M-CMTC, who had giant atrial septal aneurysm and atrial septal defect. Cerebral alterations determined by MRI were bilateral prominent lateral ventricles, bilateral cortical dysplasia, cavum septi pellucidum cyst and calvarial hemangioma. At 17th day of his life he suddenly developed cardiorespiratory arrest and died.     

Cardiac ultrasonography in structural abnormalities and arrhythmias. Recognition and treatment.

Fetal cardiac ultrasonography has become an important tool in the evaluation of fetuses at risk for cardiac anomalies. It can both guide prenatal treatment and assist the management and timing of delivery. We recommend that a fetal echocardiogram be done when there is a family history of congenital heart disease; maternal disease that may affect the fetus; a history of maternal drug use, either therapeutic or illegal; evidence of other fetal abnormalities; or evidence of fetal hydrops. The optimal timing of evaluation is 18 to 22 weeks'' gestation. An entire range of structural cardiac defects can be visualized prenatally, including atrioventricular septal defect, ventricular septal defect, cardiomyopathy, ventricular outlet obstruction, and complex cardiac defects. The outcome for a fetus with a recognized abnormality is unfavourable, with less than 50% surviving the neonatal period. Fetal cardiac arrhythmias are also a common occurrence, 15% in the series described here. Premature atrial or ventricular contractions are most commonly seen and usually require no treatment. Supraventricular tachycardia can result in hydrops and require in utero treatment to prevent fetal demise. Complete heart block, particularly in association with structural heart disease, has a poor prognosis for fetal survival.     

Shunt dynamics in experimental atrial septal defects.

Inorder to study the hemodynamic variables involving the magnitude, direction, and timing of phasic shunt flow, both the interatrial pressure gradient and blood flow along with other pertinent hemodynamic variables were measured instantaneously across a surgically created atrial septal defect (ASD) in seven awake dogs. Atrial and ventricular pacing and infusion of phenylephrine and isoproterenol were used to alter hemodynamic conditions. The wave form of phasic ASD flow was similar both in configuration and timing to the interatrial pressure gradient. During the cardiac cycle, both left-to-right (L-R) and right-to-left (R-L) shunting occurred: atrial contraction augmented L-R flow; the onset of ventricular contraction was associated with R-L flow; during the latter part of ventricular contraction, flow returned to L-R with the maximum L-R shunting occurring in early diastole. Tachycardia, infusion of phenylephrine and isoproterenol did not alter the phasic flow pattern. Both spontaneous and positive pressure respiration decreased net L-R shunting.     

Morphodynamics of flow through sinuous curvatures of the heart

Through cardiac looping during embryonic development, human and other vertebrate hearts adopt sinuous curvatures with marked changes in direction of flow at atrial, ventricular and arterial levels. We used magnetic resonance phase velocity mapping to study flow through the hearts of resting volunteers, and Doppler ultrasound to record changes with exercise. We found asymmetric recirculation of blood during filling phases of all four heart cavities, with blood redirected appropriately for onward passage to the next cavity. Doppler traces showed that biphasic ventricular filling became rapid and monophasic on strenuous exercise. We propose that looped curvatures of the heart have fluidic and dynamic advantages. Intra-cavity flow appears to be asymmetric in a manner that preserves stability, and allows momentum of inflowing streams to be redirected towards rather than away from the next cavity. Direction-change at ventricular level is such that recoil away from ejected blood is in a direction that can enhance rather than inhibit ventriculo-atrial coupling. These factors may combine to allow a reciprocating, sling-like, 'morphodynamic' mode of action become effective when heart rate and output increase with exercise. Dynamic efficiency of the looped heart may have favoured evolution of large, complex, active species characteristic of the vertebrate line.     

经胸超声心动图引导下行房间隔缺损封堵术治疗先天性房间隔缺损的临床研究

目的:探讨经胸超声心动图引导下行房间隔缺损封堵术治疗先天性房间隔缺损(Atrial septal defect,ASD)的临床疗效。方法:比较先天性ASD患者行超声心动图组(49例)或介入组(53例),患者的疗效及心脏功能的变化。结果:超声心动图组并发症发生率显著低于介入组(P0.05);术后4周,两组患者的心率、舒张期室间隔厚度(Interventricular septal thickness,IVST)、左室后壁厚度(Left ventricular posterior wall thickness,LVPWT)、左心室心肌重量(Left ventricular mass,LVM)和左心室心肌重量指数(Left ventricular mass index,LVMI)明显降低(P0.05),左心室射血分数(Left ventricular ejection fraction,LVEF)和左心室高峰充盈率(Left ventricular peak filling rate,LVPFR)均显著升高(P0.05),其余指标则无明显变化(P0.05);但术后1周超声心动图组的LVEF、IVST和LVMI即显著高于术前(P0.05)。结论:胸超声心动图引导下行ASD封堵术与X线介入封堵术疗效相当,但前者可能对ASD患者的心脏功能的改善更为显著。     

Monitoring the Function of the Right Ventricle of the Heart When Correcting an Interventricular Septal Defect

A clinical study of intracardiac hemodynamics was conducted on 46 patients with an interventricular septal defect. The practical significance of determining the contractile state of the right ventricular myocardium was confirmed based on information obtained during the long-term catheterization of the right chambers of the heart, when constructing the right ventricular function curves, and analyzing the pressure–volume diagrams. This approach to the analysis of intracardiac hemodynamic parameters versus conventional hemodynamic monitoring allows the cardiac function to be assessed on a real-time basis, the causes of the development of a low cardiac output to be revealed, and optimal ways of the regulation of myocardial contractility to be elaborated.     

SERCA2a, phospholamban, sarcolipin, and ryanodine receptors gene expression in children with congenital heart defects

In animal models of conotruncal heart defects, an abnormal calcium sensitivity of the contractile apparatus and a depressed L-type calcium current have been described. Sarcoplasmic reticulum (SR) Ca(2+) ATPase (SERCA) is a membrane protein that catalyzes the ATP-dependent transport of Ca(2+) from the cytosol to the SR. The activity of SERCA is inhibited by phospholamban (PLN) and sarcolipin (SLN), and all these proteins participate in maintaining the normal intracellular calcium handling. Ryanodine receptors (RyRs) are the major SR calcium-release channels required for excitation-contraction coupling in skeletal and cardiac muscle. Our objective was to evaluate SERCA2a (i.e., the SERCA cardiac isoform), PLN, SLN, and RyR2 (i.e., the RyR isoform enriched in the heart) gene expression in myocardial tissue of patients affected by tetralogy of Fallot (TOF), a conotruncal heart defect. The gene expression of target genes was assessed semiquantitatively by RT-PCR using the calsequestrin (CASQ, a housekeeping gene) RNA as internal standard in the atrial myocardium of 23 pediatric patients undergoing surgical correction of TOF, in 10 age-matched patients with ventricular septal defect (VSD) and in 13 age-matched children with atrial septal defect (ASD). We observed a significantly lower expression of PLN and SLN in TOF patients, while there was no difference between the expression of SERCA2a and RyR2 in TOF and VSD. These data suggest a complex mechanism aimed to enhance the intracellular Ca(2+) reserve in children affected by tetralogy of Fallot.     

Untersuchungen über die Verteilung der ABO-Blutgruppen bei angeborenen Herzfehlern

Summary This paper summarizes the own investigations in ABO blood groups in 2427 propositi with congenital heart diseases. The complete material as well as the several types valvular aortic stenosis (n=242); coarctation of the aorta (n=127); pulmonary valvular stenosis (n=211); patent ductus arteriosus (n=325); atrial septal defect (n=296); ventricular septal defect (n=612); Fallot's tetralogy (n=316) are compared with the distribution of ABO blood groups in a sample of healthy inhabitants of Süd-Niedersachsen (n=694, control I) and 81985 persons of Germany (control II). For statistical evaluation the method of Woolf is used. The relative incidence are not significant in the different proofs that means there are no statistical correlations between ABO blood groups and congenital heart disease.     

Emotional problems in children; the uses of drugs in therapeutic management

The repair of intracardiac defects under direct vision by opening the heart to expose the operative field, with the aid of hypothermia or a pump-oxygenator, is now a practical clinical method. Twelve patients were operated upon by this method. In eight patients an atrial septal defect was repaired during total circulatory occlusion under hypothermia. The seven patients in this group who had uncomplicated atrial defects survived the operation and are doing well after a short follow-up period. One patient with an unrecognized, associated ventricular defect died at the time of operation. Four patients were operated upon during total cardiac by-pass with the DeWall bubble-oxygenator. The first three patients survived operation and are continuing to do well after a brief follow-up period. In the fourth patient an atrioventricularis communis was repaired by the reconstruction of an atrial and ventricular septum with a plastic prosthesis. This patient died at the end of operation.     

Intracardiac septation requires hedgehog-dependent cellular contributions from outside the heart

Septation of the mammalian heart into four chambers requires the orchestration of multiple tissue progenitors. Abnormalities in this process can result in potentially fatal atrioventricular septation defects (AVSD). The contribution of extracardiac cells to atrial septation has recently been recognized. Here, we use a genetic marker and novel magnetic resonance microscopy techniques to demonstrate the origins of the dorsal mesenchymal protrusion in the dorsal mesocardium, and its substantial contribution to atrioventricular septation. We explore the functional significance of this tissue to atrioventricular septation through study of the previously uncharacterized AVSD phenotype of Shh(-/-) mutant mouse embryos. We demonstrate that Shh signaling is required within the dorsal mesocardium for its contribution to the atria. Failure of this addition results in severe AVSD. These studies demonstrate that AVSD can result from a primary defect in dorsal mesocardium, providing a new paradigm for the understanding of human AVSD.