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The article discusses the possible diagnostic errors in the hypoplastic left heart syndrome (HLHS) with mitral atresia and intact interventricular septum. “Atrioventricular canal defects” can be commonly and mistakenly diagnosed the prenatal period in such cases. The aspects and possibilities of differential diagnosis of these defects in the prenatal period are discussed. Ultrasound sign is presented in the article, we have named it “hockey stick with puck” that characterizes the retrograde blood flow in the aortic arch. This sign is diagnosed by color Doppler mapping of the sagittal section of the ductus arteriosus and can serve as a marker of mitral/aortic atresia.

A 35-year old man sustained fracture of cervical vertebrae in an automobile accident and pre-operative routine echocardiography showed double outlet right ventricle (DORV) with infundibular and valvar pulmonary stenosis, mitral atresia, hypoplastic left ventricle, intact ventricular septum, dysplastic tricuspid valve, secundum atrial septal defect and L-malposition of great vessels. Functionally univentricular hemodynamics allowed survival in adulthood.

Funding Acknowledgements Type of funding sources: Public Institution(s). Main funding source(s): None Previous studies have shown that an increase of epicardial adipose tissue (EAT) volume is an independent risk factor of atrial fibrillation (AF) occurrence. However, there is no reliable data about the relationship between EAT and AF recurrence after catheter ablation (CA). Also, there are no studies of the possibility of using of CT radiomics of EAT, in particular of the quantitative assessment of EAT textural changes, for prognosis of CA outcomes in patients with AF. Thus, the aim of the present study was to estimate the association of CT-radiomics features of EAT with probability of AF recurrence after catheter ablation. Materials and Methods The prospective research included 46 patients (42 males and 4 females, mean age 42.4 ± 9.36) with drug-refractory lone AF referred for catheter ablation (CA). Before CA all patients underwent multislice CT-angiography for preoperative evaluation of cardiac and vessels anatomy and volumes. Images were acquired using a 64-detector CT scanner (GE Discovery NM/CT 570c, GE Healthcare, Milwaukee, WI, USA). Imaging parameters included a gantry rotation time of 400 ms, tube voltage of 120 mA, slice thickness 1.25 mm. For evaluation of EAT only native images (contrast-free scans) without ECG synchronization were analyzed. Epicardial adipose tissue segmentation was performed by 3D-Sliser software and the SliserRadiomics module (version 4.10.2). From CT images we quantified EAT volume and 93 radiomic features, including subgroups of first-order statistics, GLCM, GLDM, GLRLM, GLSZM and NGTDM parameters. All patients were followed-up prospectively for 12 months after the CA. A blanking period of 3 months was applied. The criteria of AF recurrence were AF episodes of more than 30 sec duration. Results. Recurrence of AF was registered in 26 patients. After the end of the follow-up, we divided study population on those with (Group 1) and without (Group 2) AF recurrence. EAT volume and attenuation values for Group 1 were 176.6 ± 56.9 sm3 and -77.47 ± 2.2 HU respectively; for Group 2 were 174.05 ± 73.3 sm3 and -78.42 ± 3.3 HU respectively, with no significant differences (p In the same time, 16 of 93 CT radiomics EAT parameters were significantly different between Group 1 and Group 2 and were significantly associated with AF recurrence after CA according to univariable logistic analyses. Multivariate regression analysis demonstrated that only Gray Level Non-Uniformity Normalized (GLNUN of GLSZM) parameter was an independent predictor of AF recurrence (Odds ratio 1.0022, 95%Cl 1.0006 to 1.0038, p = 0.0013); ROC-curve analysis data showed that GLNUN > 1227.2 indicates high probability of AF recurrence during 12 months (sensitivity 84.2 %, specificity 70.8 %, AUC:0.765; p = 0.001). Conclusion radiomic biomarkers of EAT have a potential to serve as a predictors of AF recurrence after CA.

We present the case of a 1-day-old newborn, with prenatal diagnosis of Trisomy 18 and complex congenital heart disease. Echocardiography at birth showed double outlet right ventricle with non-committed interventricular communication in the setting of mitral atresia, hypoplastic left ventricle, and patent aortic root with bicuspid aortic valve and type A interrupted aortic arch. Adding anomalies were the typical congenital polyvalvular disease, Chiari network, and left intra-atrial shelf dividing morphologically left atrium. This is a rare combination of cardiac anatomical malformations in Trisomy 18.

Hypoplastic left heart syndrome (HLHS) represents a spectrum of abnormalities which marks the underdevelopment of left side cardiac structures. We present the ultrasound and autopsy correlation of two cases with HLHS with core pathology as isolated mitral atresia in one and aortic atresia in another. In both the cases after diagnostic confirmation on ultrasound (US), the couple opted for termination of pregnancy and consented for autopsy. The correlation of US and autopsy images of HLHS, facilitated our understanding of pathology behind the image obtained on US. The autopsy not only confirmed the antenatal diagnosis, but also added new findings.

An 8-month-old child presented with recurrent episodes of cyanosis and feed intolerance, progressively increasing for the past 4 months. Blood oxygen saturation was 73%. Echocardiography revealed a...

Hypoplastic Left Heart Syndrome (HLHS) represents a rare disease but still common mostly in Middle East and East world population. A large fenotype spectrum, ranging from the less severe mitral and aortic stenosis form to the more severe aortic and mitral atresia one, encompass the disease. We present here a clinical case of aortic and mitral atresia fenotype and show an operative image of the extreme form of that disease. A baby 2 d.o. 2.97 Kg has been admitted with an antenatal diagnosis of Hypoplastic Left Heart Syndrome to our Centre.

This chapter includes types, morphology, clinical manifestations, diagnosis, natural and unnatural course, treatment, and fetal diagnosis of hypoplastic left heart syndrome, including hybrid procedure, Fontan operation, and heart transplantation.

Hypoplastic left heart syndrome (HLHS) is one of the most common prenatally diagnosed congenital heart diseases because it usually profoundly alters the four-chamber view. There is a high risk of associated chromosomal, cardiac, and extracardiac defects. HLHS is associated with poor survival rates and quality of life in survivors.

We present the case of a neonate who was successfully treated with emergency hybrid palliation soon after birth, including a stent insertion into a severely thickened atrial septum and bilateral pulmonary artery banding. The patient was prenatally diagnosed with severe pulmonary venous hypertension owing to mitral atresia and intact atrial septum associated with functional single ventricle and coarctation of the aorta.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P

Mitral atresia is commonly seen as a part of the spectrum of hypoplastic left heart syndrome, and it is usually associated with multiple levels of systemic outflow tract obstruction. Isolated mitral atresia with a normal aortic valve is extremely rare. We report the rare combination of mitral atresia, transposition of the great arteries, and unobstructed systemic and pulmonary blood flow.

Background: Kilohertz frequency spinal cord stimulation (KHFSCS) is an emerging therapy for treating refractory neuropathic pain. Although KHFSCS has the potential to improve the lives of patients experiencing debilitating pain, its mechanisms of action are unknown and thus it is difficult to optimize its development. Therefore, the goal of this study was to use a computer model to investigate the direct effects of KHFSCS on specific neural elements of the spinal cord. Methods: This computer model consisted of two main components: (1) finite element models of the electric field generated by KHFSCS and (2) multicompartment cable models of axons in the spinal cord. Model analysis permitted systematic investigation into a number of variables (e.g., dorsal cerebrospinal fluid thickness, lead location, fiber collateralization, and fiber size) and their corresponding effects on excitation and conduction block thresholds during KHFSCS. Results: The results of this study suggest that direct excitation of large-diameter dorsal column or dorsal root fibers require high stimulation amplitudes that are at the upper end or outside of the range used in clinical KHFSCS (i.e., 0.5 to 5 mA). Conduction block was only possible within the clinical range for a thin dorsal cerebrospinal fluid layer. Conclusions: These results suggest that clinical KHFSCS may not function through direct activation or conduction block of dorsal column or dorsal root fibers. Although these results should be validated with further studies, the authors propose that additional concepts and/or alternative hypotheses should be considered when examining the pain relief mechanisms of KHFSCS.

Hypoplastic left ventricle, also termed hypoplastic left heart syndrome, is often associated with a variety of deformities, such as AV and aortic arch anomalies. The specimens presented in this chapter are limited to show mitral atresia and mild hypoplastic LV.

The concept of univentricular heart moved from hearts with only one ventricle connected with atria [double inlet ventricle or absent atrioventricular (AV) connection] to hearts not amenable to biventricular repair, namely hearts with two ventricles unable to sustain separately pulmonary and systemic circulations in sequence. In the latter definition, even hearts with one hypoplastic ventricle are considered “functional” univentricular hearts. They include pulmonary/aortic atresia or severe stenosis with hypoplastic ventricle, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of right ventricular cavity. In this setting, the surgical repair is univentricular with “Fontan” operation, bypassing the ventricular mass. In other words, functionally univentricular heart is a condition in which, after surgery, only one ventricle sustain systemic circulation. Univentricular hearts (double inlet or absent AV connection) almost invariably show two ventricular chambers, one main and one accessory, which lacks an inlet portion. The latter is located posteriorly when morphologically left and anteriorly when morphologically right. As far as double inlet left ventricle, this is usually associated with discordant ventriculo-arterial (VA) connection (transposition of the great arteries) and all the blood flow to the aorta, which takes origin from the hypoplastic anterior right ventricle, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet left ventricle may be rarely associated with VA concordance (Holmes heart). As far as double inlet right ventricle with posterior hypoplastic left ventricular cavity, ventriculo-arterial connection is usually of double outlet type; thus the term double inlet–outlet right ventricle may be coined. Absent right or left AV connection may develop in the setting of both d- or l-loop, whatever the situs. In this condition, the contra-lateral patent AV valve may be either mitral or tricuspid in terms of morphology and the underlying ventricle (main chamber) either morphologically left or right. Establishing the loop, whatever right or left (also called right or left ventricular topology), is a fundamental step in the segmental-sequential analysis of congenital heart disease.

Despite improvements in survival of patients with hypoplastic left heart syndrome (HLHS) with various palliative procedures, certain risk factors, such as weight less than 2.5 kg, continue to predict increased mortality. We report the palliation of a patient with HLHS weighing 1.4 kg via a hybrid procedure consisting of banding of the pulmonary arteries bilaterally, stenting the ductus arteriosus, and balloon atrial septostomy. We propose that this may be another alternative for palliation in this high-risk patient group.

[Figure][1] [![Graphic][3] ][3][![Graphic][4] ][4] A newborn was diagnosed as having hypoplastic left heart syndrome with mitral atresia. On echocardiography, 4 pulmonary veins were seen connecting to the left atrium (LA). Color flow Doppler also showed a vessel that originated

Mitral atresia is a rare congenital heart defect when compared to atresia of other valves and it is often associated with other cardiac malformation such as patent ductus arteriosus, coarctation of the aorta, transposition of the great arteries, pulmonary stenosis, shunt defects, cor triatriatum, chamber, and valvular anomalies. There have been few reports of mitral atresia, therefore, the case of a 5-month-old male child with complaint of difficulty with breathing and echocardiographic diagnosis of atretic mitral valves, atrial septal defect of 15 mms, ventricular septal defect (VSD) of 10 mms and hypoplastic left ventricle is reported.

In this Chapter only congenital malformations of the mitral valve are considered, excluding atrioventricular septal defects, which are separately treated (see chapter “Atrioventricular septal defects”), as well as mitral atresia (see chapter “Hypoplastic left heart syndrome”).

Dr Gaynor: So what shunt, what operation would you do for that patient? Dr Photiadis: Right now I think there is no data. Our data do not show it, and there are no other data, so we would still use the B-T shunt. Dr. S. Sano (Okayama, Japan): Yes, a Sano would be probably a reasonable alternative. Dr Gaynor: That is the one finding that did come out in your studies. Dr E. Bove (Ann Arbor, MI, USA): I enjoyed your paper, and I agree with your conclusion, but I rise to support what Dr. Gaynor said, and I think that we have to be very careful how we interpret data. It is a retrospective study, and if I understood from your abstract, it was surgeon preference which was not very clear. Does that mean one surgeon did one technique, another surgeon did a different one so that each surgeon decided preoperatively what he felt would be the best shunt? Additionally, looking at postoperative risk factors and showing that the Aristotle scores are equal does not have the same validity as a prospective randomized trial to reach the conclusion as you have reached. So even though I would like to agree with your conclusion, I think we have to be very careful how we interpret these data. Dr Photiadis: You are absolutely right, but again, randomisation may not give you the whole answer. If we have a patient, say aortic atresia, mitral atresia, who is stable preoperatively and whom you randomly assign to the Sano shunt, and then you have another patient with aortic atresia and mitral atresia, same anatomic type, was not prenatally diagnosed, decompensation without closure of the duct and cannot be stabilised, if you look on just the anatomic type, they are the same, but with respect to preoperative risk in fact they are not. By means of the Aristotle score these differences can be detected, with one patient being assigned to the low-risk and the other one to the high-risk group.

Interruption of the aortic arch and hypoplastic left heart syndrome in the same patient is exceptional. In the combined collections of the Registry of Cardiovascular Disease (St. Paul, Minnesota, U.S.A.) and the Registry of Congenital Heart Defects of the Rijksuniversiteit Gent (Gent, Belgium) three specimens were found with this unusual combination. These cases are herein described and compared with four similar cases previously reported in the literature.

This report briefly describes two cases of levoatriocardinal vein, in the setting of mitral atresia and a restrictive interatrial shunt, with clinical as well as echocardiographic features mimicking obstructive total anomalous pulmonary venous connection. One patient died of pulmonary hypertensive crisis, and the other survived the palliative procedures including the atrioseptectomy and pulmonary artery banding. The echocardiography, cine-angiocardiography, and magnetic resonance image for such an anomalous channel are presented. It is tempting to speculate that the thrombocytopenia and indirect hyperbilirubinemia be associated with the destructive consumption of thrombocytes and red blood cells in the serpentine levoatriocardinal vein.

A case of a previously unreported anomaly is presented in which absence of the aortic valve cusps, mitral atresia, a normal left ventricle, and an intact ventricular septum were diagnosed by cross sectional echocardiography. The development of a normal left ventricle, rather than the hypoplastic ventricle usually associated with mitral atresia, is explained by filling of the ventricular cavity via the regurgitant aortic valve.

Clinical, catheterization, and pathologic findings were recorded in a newborn infant with tuberous sclerosis and multiple cardiac rhabdomyomas that produced a clinical picture simulating mitral atresia and the hypoplastic left-heart syndrome. The clinical picture was due to a left atrial tumor that completely obstructed the mitral valvular orifice. Even if the diagnosis of left atrial tumor had been made, successful surgical correction was unlikely because of left ventricular rhabdomyomas, which produced severe subvalvular aortic stenosis and did not appear to be resectable. This case demonstrates the possibility that a hamartoma, such as a rhabdomyoma, occasionally can mimic the hypoplastic left-heart syndrome.

Three cases of persistent ostium primum coexisting with atresia of an atrioventricular valve (tricuspid atresia in one case and mitral atresia in two cases) are described. The presence of a persistent ostium primum in association with an atretic atrioventricular valve allows easy escape of blood from the atrium with an atretic valve. To our knowledge, this association of anomalies has not been previously reportd.

A case is described of mitral atresia, patent foramen ovale, subcristal ventricular septal defects (VSD), and dextro-transposition of the great arteries (d-TGA), coexisting with a normal-sized left ventricle and normal-sized great arteries in a 6 8/12-year old girl. The discussion is related to anatomic, embryologic, and surgical aspects of this particular condition, which, to our knowledge, has never been documented before.

The aortas in 41 specimeans of aortic atresia (ten with associated mitral atresia) were studied for the presence of coarctation of the aorta. The basic lesion, causing varying degrees of obstruction, was observed in 31 instances (75%), 24 minor, seven with major degrees of obstruction. In half, the aortic lesion lay proximal to the ductus, and in the majority of the remainder, opposite the ductus arteriosus. The location of lesion and degree of obstruction was essentially the same whether aortic atresia occurred alone or in association with mitral atresia. Angiography is a fairly reliable method of identifying coarctation associated with aortic atresia. An additional point of interest was that in aortic atresia alone there was strong dominance of the male sex, while this tendency was not observed when mitral atresia was associated with aortic atresia.

SUMMARY A comparison of the antiarrhythmic effects of 2 mg/kg of mepivacaine and lignocaine, as measured by elevations in the end-diastolic ventricular stimulation threshold (EDVST), was made in open-chest dogs. Both mepivacaine and lignocaine increased the EDVST significantly. The peak elevation of EDVST occurred within 2 minutes after injection and averaged 0.013 mA (13 μA) with mepivacaine and 0.022 mA (22 μA) with lignocaine. The greater early response to lignocaine as compared with mepivacaine was significant. After 4 minutes the rate of decreasing effect was approximately 0.001 mA/min for both mepivacaine and lignocaine. The studies provide new evidence demonstrating the antiarrhythmic properties of mepivacaine and support the continued evaluation of related compounds in the search for better antiarrhythmic agents.

1. 1. Thirty-three cases of tricuspid atresia and twenty-eight cases of mitral atresia have previously been reported. 2. 2. We report one additional ease of tricuspid atresia associated with transposition of the aorta and atresia of the pulmonary artery, and one additional ease of mitral atresia associated with complete transposition of the great vessels. Other anomalies were present in both of these cases. No cases were found in the literature in which there were these same combinations of defects. 3. 3. The clinical and embryologic aspects of each of these lesions are discussed.

A case of congenital mitral and aortic atresia, associated with hypoplasia of the left atrium and ventricle and compensating patencies of the interatrial foramen and ductus arteriosus, is described. The anomaly is attributed to a developmental misplacement of the interventricular septum. No evidence was found to indicate an inflammatory cause for the defect.

A newborn is presented with an association of hypoplastic left heart syndrome, cor triatriatum and partial anomalous pulmonary venous connection. The diagnosis was established with echocardiography and further confirmed with computed tomography. To our knowledge the images of such an association have never been reported before. © 2011 .

The morphological characteristics of mitral atresia were studied in 30 hearts to determine the presence or absence of a morphological rudiment of the atretic valve and the relation of this rudiment, if found, to any chamber in the ventricular mass. All the hearts showed atrial situs solitus and no ventricular inversion; consequently all had left atrioventricular atresia. In all instances dense fibrous tissue connected the floor of the left atrium to the left ventricle. This connective tissue is considered to be the morphological rudiment of the atretic mitral valve. In several hearts the intervening fibrous tissue varied from a thick fibrous membrane to a tiny fibrous cord; it is impossible to detect these variations clinically. It is, therefore, more practical to classify those hearts which have a detectable fibrous membrane macroscopically as having an "imperforate membrane" and those with a fibrous strand detectable only microscopically as having an "absent atrioventricular connection."

A heart specimen is presented which showed a rare combination of atrioventricular valve atresia with single ventricle, truncus arteriosus communis with an anteriorly placed trunk, ie transposition. Because of the problems involved in the classification of this heart, the types of single ventricle are revised with particular attention to the rare cases of atrioventricular valve atresia and single ventricle reported in the literature. Also the rare type of truncus arteriosus communis with truncoatrioventricular valve discontinuity is excluded from the so-called transposition complexes, and properly classified as a form of truncus. The literal meaning of transposition is stressed and it is urged to approach congenital heart defects in a purely anatomic sense with definition of each segment as accurately as possible. Thus the present confusion in terminology and further plethora of new terms are avoided.

Eighteen cases of mitral atresia with normal aortic valve plus 68 cases from the literature are analyzed. A new classification based on anatomical findings is proposed. Pulmonary stenosis or atresia is frequent in the type with transposition of the great arteries. The rare instances of normal or large left ventricle are due to a large ventricular septal defect, or to straddling or displaced tricuspid valve. The clinical, radiologic and electrocardiographic findings have been summarized and correlated with the different physiopathological situations. In our catheterized cases an oxygen saturation step-up was found in the right atrium together with left atrial hypertension. In seven cases mitral atresia was demonstrated by selective left atrial contrast injection. The average age at death was six months. Among the cases surviving one year or more, the association of atrial septal defects or pulmonary stenosis was frequent. Enlargement of the interatrial communication, accompanied by other palliative measures, is the only available surgical procedure.

Hypoplastic left heart syndrome (HLHS), a term used to describe severe underdevelopment of left heart structures, is characterized by aortic valve atresia and hypoplastic left ventricle, and may include mitral atresia and/ or hypoplastic segments of the aorta. This syndrome accounts for 2% of all congenital heart disease, or approximately 350 infants annually in the United States [1]. Without treatment, 100% of these infants die before 1 year of age; the vast majority succumb within the first month of life. The plight of infants with this syndrome received national attention late in 1984, when an affected child was the recipient of the heart of a baboon; a procedure that kept her alive for 3 well-publicized weeks.

This is a study of the course of the conduction system in two cases of hypoplasia of the aortic tract complex, one with mitral stenosis and the other with mitral atresia. In both there was a posterior atrioventricular (AV) node which formed the AV bundle. In case 1 the bundle was short and bifurcated early. The branching bundle gave off a large left bundle branch (LBB), many Mahaim fibers to the septum and a small right bundle branch (RBB). In case 2 the AV node was divided into two parts which formed two posterior bundles which joined together to form a short branching bundle. Instead of a LBB there were profuse Mahaim fibers passing from the branching bundle to the depths of the ventricular septum. The RBB was large. The abnormalities seen in the conduction system particularly in the LBB are discussed from the embryologic stand-point.

The clinical picture of tricuspid or mitral atresia may be due either to absence of an atrioventricular connexion or to the presence of an imperforate membrane [1]. The distinction between these types may in itself have clinical significance and for this cross-sectional two-dimensional (2D) echocardiography should provide the ideal tool. In this study we have investigated 46 patients known to have right or left atrioventricular valve atresia, and correlated our 2D echocardiographic findings with a study of similar autopsy material.

Three cases of mitral atresia with overriding right atrioventricular valve, ventricular septal defect, and normally sized left ventricle are reported, two of them with transposition of the great vessels. Although the combination of anomalies is rare, the possibility that palliative and/or corrective surgery could be accomplished indicates that in suspected cases of mitral atresia, diagnostic efforts aimed at accurate delineation of the associated defects must be made.

The effects of methylprednisolone (50 mg.kg-1) on the incidence of ventricular tachycardia and fibrillation and on ventricular fibrillation threshold were studied during acute coronary occlusion in anaesthetised dogs. Ventricular tachycardia and/or ventricular fibrillation occurred in 11 of the 16 animals (69%) both before and after methylprednisolone pretreatment. The mean ventricular fibrillation threshold of 10 dogs was 10.1 +/- 1.8 mA before methylprednisolone and it increased slightly to 13.3 +/- 2.3 mA after the drug. This difference was not statistically significant (P greater than 0.2).

From a survey of newborn infants using truncate ascertainment of families through affected offspring, 64 cases of hypoplastic left heart syndrome (HLHS) were found. Pedigree information was obtained from 42 families. The incidence of HLHS, based on 64 affected infants, was 36.5/100,000 births. The incidence of HLHS with aortic and/or mitral atresia was 25.1/100,000 births. The recurrence among later siblings was about 2 %. Autosomal recessive hypothesis of transmission of this disorder was not supported. Using Edwards' formula, the observed recurrence among later siblings was compatible with polygenic inheritance.

We describe a 4-week-old baby with mitral atresia, hypoplastic left ventricle, ventricular septal defect, preductal coarctation and premature closure of the oval foramen whose only outlet from the left atrium was a stenosed right-sided levoatriocardinal vein and who in addition developed left atrial thrombi. Cross-sectional echocardiography was extremely helpful in establishing the diagnosis.