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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.

Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended. [ABSTRACT FROM AUTHOR]

A 5-year-old female alpaca was presented with respiratory distress and lethargy. Thoracic radiographs revealed a cranioventrally distributed alveolar pattern, caudodorsal bronchial pattern, cardiomegaly, increased soft tissue opaque content in the ventral thorax, and rounded soft tissue opaque structures craniodorsal to the carina. Cardiac gated CT demonstrated a patent ductus arteriosus, ventricular septal defect, complete left atrioventricular valve atresia, partial anomalous venous connections from the cranial pulmonary veins to the azygous and cranial vena cava, severe right-sided cardiomegaly, pleural and peritoneal fluid, and severe hepatic congestion. These findings were confirmed with necropsy., (© 2024 American College of Veterinary Radiology.)

Aim: To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. Materials and Methods: We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. Results: A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo‐arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. Conclusions: Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia. [ABSTRACT FROM AUTHOR]

As yet, there is no agreed definition for the so-called “hypoplastic left heart syndrome”. Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a “syndrome” in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described “hypoplasia of the aortic outflow tract complex”. In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.

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.

Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly affecting the left ventricle, associated valves, and ascending aorta. Although in clinical practice HLHS is usually sub-categorised based on the patency of the mitral and aortic (left-sided) valves, it is also possible to comprehensively categorise HLHS into defined sub-groups based on the left ventricular morphology. Here, we discuss the published human-based studies of the ventricular myocardium in HLHS, evaluating whether the available evidence is in keeping with this ventricular morphology concept. Specifically, we highlight results from histological studies, indicating that the appearance of cardiomyocytes can be different based on the sub-group of HLHS. In addition, we discuss the histological appearances of endocardial fibroelastosis (EFE), which is a common feature of one specific sub-group of HLHS. Lastly, we suggest investigations that should ideally be undertaken using HLHS myocardial tissues at early stages of HLHS development to identify biological pathways and aid the understanding of HLHS aetiology.

Mitral atresia with normal aortic root is a rare complex congenital heart defect (CHD) and constitute less than 1% of all CHDs. In this anomaly, the mitral valve is atretic, a patent foramen ovale provides egress of the left atrial blood, either a single ventricle or two ventricles with left ventricular hypoplasia are present, and the aortic valve/root are normal by definition. Clinical, roentgenographic and electrocardiographic features are non-distinctive, but echo-Doppler studies are useful in defining the anatomic and pathophysiologic components of this anomaly with rare need for other imaging studies. Treatment consists of addressing the pathophysiology resulting from defect and associated cardiac anomalies at the time of initial presentation, usually in the early infancy. These children eventually require staged total cavo-pulmonary connection (Fontan) in three stages. Discussion of each of these stages were presented. Complications are observed in-between the stages of Fontan surgery and following completion of Fontan procedure. Attempts to monitor for early detection of these complications and promptly addressing the complications are recommended.

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. The complete cohort ultimately gathered 32 cases described in the literature completed by seven cases from our unit (seven fetuses and one child). Four types of univentricular hearts associated with CAT were observed: tricuspid atresia or hypoplastic right ventricle in 16 cases, mitral atresia or hypoplastic left ventricle in 12 cases, double-inlet left ventricle in 2 cases, and unbalanced atrioventricular septal defect in 9 cases. Our study questions the diagnosis of CAT as the exclusive consequence of an anomaly of the wedging process, following the convergence between the embryonic atrioventricular canal and the common outflow tract. We confirm that some forms of CAT can be considered to be due to an arrest of cardiac development at the stages preceding the convergence.

Background Transcranial electrical stimulation has broad potential as a treatment for depression. Transcranial random noise stimulation, which delivers randomly fluctuating current intensities, may have greater cortical excitatory effects compared with other forms of transcranial electrical stimulation. We therefore aimed to investigate the antidepressant efficacy of transcranial random noise stimulation. Methods Depressed participants were randomly assigned by computer number generator to receive 20 sessions of either active or sham transcranial random noise stimulation over 4 weeks in a double-blinded, parallel group randomized-controlled trial. Transcranial random noise stimulation was delivered for 30 minutes with a direct current offset of 2 mA and a random noise range of 2 mA. Primary analyses assessed changes in depression severity using the Montgomery-Asperg Depression Rating Scale. Neuroplasticity, neuropsychological, and safety outcomes were analyzed as secondary measures. Results Sixty-nine participants were randomized, of which 3 discontinued treatment early, leaving 66 (sham n = 34, active n = 32) for per-protocol analysis. Depression severity scores reduced in both groups (Montgomery-Asperg Depression Rating Scale reduction in sham = 7.0 [95% CI = 5.0–8.9]; and active = 5.2 [95% CI = 3.2–7.3]). However, there were no differences between active and sham groups in the reduction of depressive symptoms or the number of participants meeting response (sham = 14.7%; active = 3.1%) and remission criteria (sham = 5.9%; active = 0%). Erythema, paresthesia, fatigue, and dizziness/light-headedness occurred more frequently in the active transcranial random noise stimulation group. Neuroplasticity, neuropsychological, and acute cognitive effects were comparable between groups. Conclusion Our results do not support the use of transcranial random noise stimulation with the current stimulation parameters as a therapeutic intervention for the treatment of depression. Clinical trial registration at clinicaltrials gov/NCT01792414. [ABSTRACT FROM AUTHOR]

Introduction: Transcranial electrical stimulation (tES) as a method of cognitive enhancement in both diseased and healthy individuals has gained popularity. Its potential for enhancing cognition in healthy individuals has gained the interest of the military. However, before it being implemented into military training or operational settings, further work is needed to determine its efficacy and safety. Although a considerable amount of literature exists, few studies have specifically evaluated its use in enhancing cognition relative to operational, military tasks. Therefore, in a first step to evaluate its efficacy, we completed a systematic literature review of studies using transcranial direct current stimulation (tDCS), a type of tES, to enhance cognitive processes in healthy individuals.Methods: A systematic literature review was conducted to identify literature published between 2008 and 2018 that used a method of tES for cognitive enhancement. As part of a larger literature review effort, 282 articles were initially retrieved. These were then screened to identify articles meeting predetermined criteria, to include those using various methods of tES, resulting in 44 articles. Next, the articles were screened for those using tDCS or high-definition tDCS, resulting in 34 articles for review and information extraction.Results: Of the 34 articles reviewed, 28 reported some degree of enhancement (eg, improved accuracy on tasks and reduced reaction times). Areas of cognitive enhancements included executive functioning, creativity/cognitive flexibility, attention/perception, decision-making, memory, and working memory. However, the precise outcomes of enhancement varied given the range in tasks that were used to assess the constructs. Additionally, the stimulation parameters in terms of intensity applied, duration of stimulation, and brain region targeted for stimulation varied.Conclusions: The conclusions to be drawn from this systematic literature review include the identification of a brain region for targeting with stimulation to enhance a broad range of cognitive constructs applicable to military tasks, as well as stimulation parameters for duration and intensity. The dorsolateral prefrontal cortex was most frequently targeted in the studies that found enhanced performance across several cognitive constructs. Stimulation intensities of 2 mA and durations of 20 minutes or longer appeared frequently as well. Although several parameters were identified, further work is required before this type of technology can be recommended for operational use. [ABSTRACT FROM AUTHOR]

Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection. Apart from the above shunts, LACV contributed to PAH in these cases. It is important to detect and report LACV as this may require surgical correction along with other defects. If LACV goes undetected during imaging workup, it may cause persistent PAH postoperatively.

Congenitally corrected transposition (ccTGA), also known as L-transposition of the great arteries (L-TGA), is a rare cardiac malformation accounting for approximately 0.05% of congenital heart disease, characterized by ventricular inversion, discordant ventriculo-arterial connections with a normal visceroatrial relationship. It was first described by Baron Rokitansky in 1875, and prenatal sonographic diagnosis can be difficult. Symptomatology and clinical presentation of this malformation are related to the associated intracardiac defects. We report a rare case of severe, complex cardiac disease: prenatally diagnosed ccTGA with atrial restriction, mitral atresia, Ebsteinoid tricuspid valve, and severe pulmonary valve stenosis, who subsequently developed left pulmonary vein stenosis. [ABSTRACT FROM AUTHOR]

In trauma, admission rapid thrombelastography (rTEG) has been shown to predict in-hospital thromboembolic events, guide treatment of coagulopathy, and identify likely to require large volume resuscitations. We sought to evaluate the use of rTEG in describing the coagulation status of major burn patients at admission and assess whether rTEG values predicted resuscitation volumes and patient outcomes. This is a retrospective study of all patients admitted to our Burn intensive care unit between January 2010 and December 2012. We excluded those with < 15% TBSA burns, < 18 years of age, and with concomitant injuries requiring admission to the Trauma intensive care unit. Previously published and validated cut points for hypocoagulable (activated clotting time ≥ 128; k-time ≥ 2.5; angle ≤ 60; mA ≤ 55; LY30 ≥ 3%) and hypercoagulable (mA ≥ 65) rTEG values were used. Supra-normal burn resuscitation was defined as ≥ 5.0 mL/kg/TBSA. Statistical analyses were conducted using STATA 13.1. Sixty-five patients met inclusion with a median age of 45 years, 74% male and 49% white. Median TBSA was 38% with 14% having third-degree burns. Sixty percentage of patients were hypercoagulable on admission, while 24% were hypocoagulable. rTEG values predicted increased 24-hour resuscitation volumes, as well as plasma and platelet transfusions (P < 0.05). Controlling for age, TBSA, and base deficit, admission rTEG ≥ 128 predicted a 5-fold increased likelihood of supra-normal resuscitation. In addition, an angle < 60 predicted in-hospital mortality. While the majority of severely burned patients arrive hypercoagulable, one-quarter are hypocoagulable and have increased resuscitation and transfusion requirements. Moreover, those with admission activated clotting time ≥ 128 are at 5-fold increased risk of supra-normal resuscitation. [ABSTRACT FROM AUTHOR]

Levoatrial cardinal vein (LACV) is anomalous connection between left atrium or pulmonary veins and systemic veins such as innominate vein or superior vena cava. This persistence of splanchnic circulation occurs when there is left-sided obstructive cardiac lesions such as hypoplastic left heart or mitral atresia. In this report we present three cases of LACV with well-developed left heart, without any obstructive lesions. All our cases presented with pulmonary arterial hypertension (PAH) and had associated intracardiac shunt such as ventricular/atrial septal defect and supracardiac partial anomalous pulmonary venous connection. Apart from the above shunts, LACV contributed to PAH in these cases. It is important to detect and report LACV as this may require surgical correction along with other defects. If LACV goes undetected during imaging workup, it may cause persistent PAH postoperatively. [ABSTRACT FROM AUTHOR]

As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a "syndrome" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described "hypoplasia of the aortic outflow tract complex". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.

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. [ABSTRACT FROM AUTHOR]

The concept of univentricular heart moved from hearts with only one ventricle connected with atria (double inlet ventricle or absent 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 or aortic atresia, severe stenosis, and rare conditions like huge intramural cardiac tumors and Ebstein anomaly with extreme atrialization of RV cavity. In this setting, the surgical repair is univentricular with Fontan operation, bypassing the ventricular mass. The cases of anatomically true 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 LV, this is usually associated with discordant VA connection (transposition of the great arteries). All the blood flow to the aorta, which takes origin from the hypoplastic anterior RV, is ventricular septal defect (bulbo-ventricular foramen) dependent. If restrictive, an aortic arch obstruction may be present. Double inlet LV may be associated with VA concordance (Holmes heart). As far as double inlet RV with posterior hypoplastic LV cavity, ventriculo- arterial connection is usually of double outlet type. Thus the term double inlet-outlet RV may be coined. 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 LV topology), is a fundamental step in the segmental-sequential analysis of congenital heart diseases.

Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly affecting the left ventricle, associated valves, and ascending aorta. Although in clinical practice HLHS is usually sub-categorised based on the patency of the mitral and aortic (left-sided) valves, it is also possible to comprehensively categorise HLHS into defined sub-groups based on the left ventricular morphology. Here, we discuss the published human-based studies of the ventricular myocardium in HLHS, evaluating whether the available evidence is in keeping with this ventricular morphology concept. Specifically, we highlight results from histological studies, indicating that the appearance of cardiomyocytes can be different based on the sub-group of HLHS. In addition, we discuss the histological appearances of endocardial fibroelastosis (EFE), which is a common feature of one specific sub-group of HLHS. Lastly, we suggest investigations that should ideally be undertaken using HLHS myocardial tissues at early stages of HLHS development to identify biological pathways and aid the understanding of HLHS aetiology.

The association of truncus arteriosus communis with interrupted aortic arch and mitral atresia is an exceptionally rare congenital defect. We describe the initial decision-making and management of this lesion, which eventually achieved a Fontan palliation.

Abstract: 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. [Copyright &y& Elsevier]

The atrioventricular (AV) conduction system in AV discordance remains unclear, especially in cases with complex cardiac anomaly. We report a case of accessory pathway reciprocating tachycardia in atrioventricular discordance (AVD) and mitral atresia with twin AV nodes. In this case, the anterior AV node was located along the atretic mitral valve. The anterior AV node was involved in tachycardia and the posterior AV node acted as a bystander during tachycardia. The anterior AV node in AVD can be located along the atretic mitral valve, and one of twin AV nodes might act as a bystander during AV reciprocating tachycardia. (PACE 2010; 637–640) [ABSTRACT FROM AUTHOR]

We report the first known case of superoinferior ventricles with a superior morphologically left ventricle and an inferior morphologically right ventricle. This 2½-year-old boy also had dextrocardia, double-outlet right ventricle {S,L,L}, right-sided mitral atresia, left-sided tricuspid regurgitation, a large conoventricular type of ventricular septal defect, and pulmonary outflow tract stenosis. This very rare form of superoinferior ventricles appears to be due to excessive levorotation (≃170°) of discordant L-loop ventricles. [ABSTRACT FROM AUTHOR]

Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-monthold boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was succesfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. The complete cohort ultimately gathered 32 cases described in the literature completed by seven cases from our unit (seven fetuses and one child). Four types of univentricular hearts associated with CAT were observed: tricuspid atresia or hypoplastic right ventricle in 16 cases, mitral atresia or hypoplastic left ventricle in 12 cases, double-inlet left ventricle in 2 cases, and unbalanced atrioventricular septal defect in 9 cases. Our study questions the diagnosis of CAT as the exclusive consequence of an anomaly of the wedging process, following the convergence between the embryonic atrioventricular canal and the common outflow tract. We confirm that some forms of CAT can be considered to be due to an arrest of cardiac development at the stages preceding the convergence.

Two cases of the hypoplastic left heart syndrome with premature closure of the foramen ovale and levoatriocardinal veins are described and the literature is reviewed. One of the patients is the only case with both mitral and aortic valve atresia. The other is associated with the asplenia syndrome, double-outlet right ventricle without ventricular septal defect, and an anomalous connection of the left atrium to the right auricle. [ABSTRACT FROM AUTHOR]

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 case previously reported in the literature. [ABSTRACT FROM AUTHOR]

The atrioventricular junction of 40 patients with univentricular heart was evaluated by two-dimensional echocardiography. The apical 4 chamber view optimally imaged the atrioventricular junction, and allowed determination of the type of atrioventricular connection: double inlet, common atrioventricular orifice, and absent right or left atrioventricular connection. When double inlet to 1 ventricle was demonstrated, the 4 chamber view allowed immediate comparison of the form and function of the right and left atrioventricular valves. Because anomalies of the atrioventricular valves frequently complicate the univentricular heart, two-dimensional echocardiographic assessment is a most important adjunct to the preoperative investigation of these patients. [ABSTRACT FROM AUTHOR]

Echocardiograms and angiograms of six infants with left ventricular hypoplasia were examined. Three observations were made: (1) A normal-sized aortic root echogram may be seen in infants with mitral atresia, intact ventricular septum and normally related great arteries. (2) A recognizable mitral valve echogram may be seen in mitral atresia. (3) The true echocardiographic left ventricular dimension may be exceedingly difficult to measure, the tendency being to overestimate it. Awareness of these pitfalls will aid in the echocardiographic diagnosis of left ventricular hypoplasia in infants. [ABSTRACT FROM AUTHOR]

Whether a chamber is or is not a ventricle is determined by its myocardial morphological characteristics, not by the entering atrioventricular (AV) valves. The anatomic characteristics of the morphologically right ventricle and the morphologically left ventricle have been well described, are widely understood, and do not need to be changed. What a ventricle is should not be redefined in terms of the AV connections because such redefinition is based on the classic definition of single ventricle, which itself is not satisfactory. This old definition is wrong in principle, violates the morphological method of diagnosis and designation of the cardiac chambers, and has two kinds of exceptions. Consequently the classic definition of single ventricle must not be used as a paradigm (model) for the redefinition of what a ventricle is or is not. The approach and terminology based on the use of the unsatisfactory single-ventricle paradigm should be discontinued, eg, 'primitive ventricle,' 'main chamber,' 'accessory chamber,' 'trabecular pouch.' Being unnecessary, these nonmorphological components of the terminology of congenital heart disease may be omitted. Morphological anatomy, unadorned, is the key to clarity, simplicity, and accuracy. [ABSTRACT FROM AUTHOR]

Thirty-five hearts were studied with no patent communication between the left atrium and the ventricular mass (‘mitral atresia’). In ten, an imperforate and hypoplastic membrane separated the left atrium from a hypoplastic left ventricle. The imperforate valve ranged from a tiny membrane with no evidence of tension apparatus, through a larger membrane supported by muscular columns in the ventricular inlet portion, to an aneurysmal imperforate sac attached to a single hypoplastic papillary muscle. The ventriculo-arterial connection was concordant in nine of the ten hearts and double outlet from the right ventricle in one. In one further heart, there was double inlet left ventricle with imperforate left atrioventricular valve, a rudimentary right ventricle and ventriculo-arterial discordance. In the other 24 hearts the muscular floor of the left atrium was completely separated by the atrioventricular sulcus from the ventricular mass (absent left atrioventricular connection). In II of these hearts, the tight atrium was connected to a right ventricle and there was a posterior and left-sided rudimentary left ventricular chamber which had no connection with the atrial chambers. The left ventricle supported the aorta in five of the II hearts, but in the other six it was simply a pouch of left ventricular morphology. In ten cases the right atrium was connected to a dominant left ventricle. An anterior rudimentary right ventricle was present in each but had no connection with the atrial chambers. It was to the left of the left ventricle in seven, directly anterior in one and to the right in two cases. The right ventricle supported the aorta in eight cases, the pulmonary trunk in one and neither great artery in the remaining case. Lastly, there were three cases in which a sole ventricular chamber of indeterminate morphology was present and gave rise to both great arteries. [ABSTRACT FROM PUBLISHER]