Double outlet right atrium in a dog.

Double outlet right atrium is a rare congenital cardiac abnormality that has been previously reported in humans and cats, but not in dogs. A double outlet right atrium is typically characterized by the presence of a leftward deviation of the interatrial septum and atrial septal defect. Therefore, the right atrium drains into both ventricles. The unique features consistent with double outlet right atrium were identified by transthoracic echocardiography and computed tomography in a puppy. This case report describes the clinical, echocardiographic, and tomographic findings of a five-month-old Cocker Spaniel diagnosed with this rare congenital abnormality.

Open-heart surgery using Del-Nido cardioplegia in two dogs: partial atrioventricular septal defect and mitral repair.

Del-Nido cardioplegia (DNc) is a single-dose cardioplegia that is widely used in human medicine because of its long duration. In this report, we describe two cases of open-heart surgery with cardiopulmonary bypass (CPB) using DNc. One dog was diagnosed with partial atrioventricular septal defect, and the other dog was diagnosed with myxomatous mitral valve disease stage D. Both dogs were treated with open-heart surgery with DNc to induce temporary cardiac arrest. No complications from DNc were observed, and the patients were discharged. Veterinary heart surgeons should consider DNc as an option for temporary cardiac arrest during open-heart surgery with CPB.

National outcomes of the Fontan operation with endocardial cushion defect.

BACKGROUND: The traditional outcomes of the Fontan operation (FO) in endocardial cushion defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect, and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings. METHODS: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data were abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. RESULTS: Three thousand three hundred eighty patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p = .04). The length of stay (16 vs. 13 days, p = .05) and total charges incurred ($283, 280 vs. $234, 106, p = .03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis, ECD diagnosis, cardiac arrest, acute kidney injury, and postoperative hemorrhage were predictors of mortality. CONCLUSION: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than in non-ECD patients. The occurrence of postoperation complications and a diagnosis of ECD were predictive of a negative outcome.

Trifoliate left atrioventricular valve with and without intact septal structures in four dogs: echocardiographic findings and surgical repair.

Trifoliate left atrioventricular (AV) valve with common atrioventricular junction is considered part of the spectrum of atrioventricular septal defect. This valve morphology is typically associated with defects in the AV septum resulting in communication at the atrial or ventricular level, but has also been described as an isolated defect in the setting of a common AV junction without AV septal defect. Trifoliate left AV valve exhibits a line of apposition between the bridging leaflets that is directed toward the inlet interventricular septum, distinguishing it from isolated mitral valve cleft in which the orientation of the bridging leaflets are toward the left ventricular outflow tract. The echocardiographic findings of four dogs with trifoliate left AV valve are described; two with intact septal structures and two with large ostium primum defects. Three dogs underwent open surgical repair using different approaches depending on the presence or absence of a septal defect. One of these underwent concurrent surgical repair for right AV valve dysplasia. One dog with intact septal structures underwent interventional closure of a concurrent patent ductus arteriosus. Current terminology associated with trileaflet left AV valve malformations is reviewed.

Association of Alcohol Use Diagnostic Codes in Pregnancy and Offspring Conotruncal and Endocardial Cushion Heart Defects.

Background The pathogenesis of congenital heart disease (CHD) remains largely unknown, with only a small percentage explained solely by genetic causes. Modifiable environmental risk factors, such as alcohol, are suggested to play an important role in CHD pathogenesis. We sought to evaluate the association between prenatal alcohol exposure and CHD to gain insight into which components of cardiac development may be most vulnerable to the teratogenic effects of alcohol. Methods and Results This was a retrospective analysis of hospital discharge records from the California Office of Statewide Health Planning and Development and linked birth certificate records restricted to singleton, live-born infants from 2005 to 2017. Of the 5 820 961 births included, 16 953 had an alcohol-related International Classification of Diseases, Ninth and Tenth Revisions (ICD-9; ICD-10) code during pregnancy. Log linear regression was used to calculate risk ratios (RR) for CHD among individuals with an alcohol-related ICD-9 and ICD10 code during pregnancy versus those without. Three models were created: (1) unadjusted, (2) adjusted for maternal demographic factors, and (3) adjusted for maternal demographic factors and comorbidities. Maternal alcohol-related code was associated with an increased risk for CHD in all models (RR, 1.33 to 1.84); conotruncal (RR, 1.62 to 2.11) and endocardial cushion (RR, 2.71 to 3.59) defects were individually associated with elevated risk in all models. Conclusions Alcohol-related diagnostic codes in pregnancy were associated with an increased risk of an offspring with a CHD, with a particular risk for endocardial cushion and conotruncal defects. The mechanistic basis for this phenotypic enrichment requires further investigation.

Echocardiographic diagnosis of special type of atrioventricular septal defect / 中华超声影像学杂志

Objective:To improve the understanding of special type of atrioventricular septal defect (AVSD) and reduce clinical missed diagnosis and misdiagnosis by investigating its echocardiographic features.Methods:Echocardiographic images of patients with special type of AVSD diagnosed by echocardiography and confirmed by surgery in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University from November 2011 to November 2021 were retrospectively analyzed. Depending on the presence of ventricular septal defect(VSD), special type of AVSD were divided into two types, namely AVSD with intact ostium primum and AVSD with intact ostium primum and ventricular septum. The echocardiographic features of special type of AVSD were summarized.Results:Twenty-five cases of special type of AVSD were diagnosed by echocardiography and 13 cases were confirmed by surgery, including 12 cases with intact ostium primum and 1 case with intact ostium primum and ventricular septum. There were 5 cases without surgical indications, and 1 case was kept under observation due to small VSD. One patient with complex malformation only underwent pulmonary artery banding operation without treating intracardiac malformation. The other 4 cases of perimembrane VSD and 1 case of partial AVSD were misdiagnosed as special type of AVSD by echocardiography. All patients had mild to severe left atrioventricular regurgitation before surgery, and most patients remained left atrioventricular regurgitation of varying degrees after left atrioventricular valve formation. Characteristic sonographical findings included: ①The ostium primum was continuous and there was no ostium primum defect. ②The attachment points of the left and right atrioventricular valves were at the same level. ③The left atvioventricular valve was a trileaflet left atrioventricular valve and attached to the crest of the ventricular septum, forming the so-called "anterior mitral valve leaflet cleft". ④The two groups of papillary muscles of the left ventricle changed the normal anterolateral and posterolateral alignment, but showed a anteriorly and posteriorly relationship (counterclockwise transposition). ⑤In the presence of VSD, an inlet ventricular septal defect would be present. ⑥Color Doppler showed varying degrees of regurgitation signals from the left atrioventricular valve during systolic period. ⑦Other complex deformities may be combined with special type of AVSD.Conclusions:Echocardiography can make a definite diagnosis for special types of AVSD. The prognosis of this malformation is closely related to the left atrioventricular valve, and the shaping effect depends on the development of left atrioventricular valve and regurgitation.

"Repair of common atrioventricular junction in isolation and when associated with other congenital heart defects".

Management of atrioventricular septal defects has grown leaps and bounds since the first correction of this malformation. Main reasons considered for the improvement are good understanding of lesion anatomy, precise preoperative diagnosis including imaging, progress in surgical technique, myocardial protection, and post-operative care. In this article we review the anatomy, pathophysiology, clinical features, associations, diagnosis, surgical therapy, complications, and results of atrioventricular septal defects.

A case of a complete atrioventricular canal defect in a ferret.

BACKGROUND: Atrioventricular canal defect is a rare congenital disorder of the heart and describes the presence of an atrial septal defect, a variable presentation of ventricular septal alterations including ventricular septal defect malformations in the mitral and tricuspid valves. The defect has been described in human beings, dogs, cats, pigs, and horses. CASE PRESENTATION: This paper describes the case of a complete atrioventricular canal defect in a four-year-old intact male pet ferret (Mustela putorius furo), which was presented due to posterior weakness, ataxia, and decreased appetite. A loud systolic murmur, dyspnea, and hind limb paraparesis were detected during the clinical examination. Thoracic radiographs showed generalized cardiomegaly and lung edema. ECG showed sinus rhythm with prolonged P waves and QRS complexes. Echocardiography showed a large atrial septal defect, atrioventricular dysplasia, and a ventricular septal defect. Palliative treatment with oxygen, furosemide, spironolactone, enalapril, diltiazem, and supportive care was chosen as the therapy of choice. The ferret recovered gradually during hospitalization. A follow-up examination at three and six months showed stabilization of cardiac function. CONCLUSIONS: To the authors knowledge, this is the first time an atrioventricular canal defect has been described in a pet ferret.

Major Septal Defects: Comparative Study of Down Syndrome and Non-Down Syndrome Infants, Before and After Surgery.

OBJECTIVE: To compare pre-operative, intra-operative, and post-operative parameters in Down syndrome (DS) and non-DS patients with atrioventricular septal defects (AVSD) and inlet ventricular septal defects (VSD) in a tertiary care hospital in Pakistan. METHODS: We conducted a retrospective study at Aga Khan University, Pakistan. All complete atrioventricular septal defect (CAVSD), partial atrioventricular septal defect (PAVSD), and VSD with inlet extension surgical cases from January 2007 to January 2019 were included. Patients with congenital heart diseases other than those listed above were excluded. RESULTS: In 61 cases, 18 had DS. Median age, mean body surface area (BSA), and height were lower in DS patients compared to non-DS patients: 7.0 vs 23.0 months, 0.311 vs 0.487 m2, and 63 vs 82 cm, respectively. Bypass duration, aortic cross clamp time, post-operative ventilator hours, dose of inotropes, CICU stay, and total hospital stay were all significantly higher in the DS group. The odds ratio (955% CI) for mortality in DS babies was 6.2 (1.4, 27.1), p=0.015, after adjusting for age, weight, and height. The overall morbidity was comparable between the two groups, demonstrating no significant difference after adjusting for confounders. CONCLUSION: DS babies with AVSD and inlet VSD are at a greater risk of mortality compared to non-DS babies, particularly those with CAVSD. Furthermore, DS babies undergo surgery at a younger age and require more aggressive post-operative therapy and monitoring due to the development of complications.

Repair of Complete Atrioventricular Septal Defects With Decellularized Extracellular Matrix: Initial and Midterm Outcomes.

OBJECTIVE: Since April 2010, our institution has repaired complete atrioventricular septal defects (CAVSDs) with a two-patch technique utilizing CorMatrix extracellular material. This material is potentially an attractive patch because of its theorized eventual integration with the host tissue. We sought to analyze initial outcomes of CAVSD repair with CorMatrix. METHODS: Data were collected on consecutive pediatric (age <18) patients receiving two-patch CAVSD repairs with CorMatrix at a single institution from April 2010 to July 2014. Baseline and perioperative characteristics were evaluated. Echocardiograms were evaluated in both the immediate postoperative period and the most recent postoperative follow-up. Variables analyzed included left AV valve performance, residual shunting, left ventricular outflow tract (LVOT) gradient, morbidity, and mortality. RESULTS: Fifteen patients were identified. The average age at operation was 205 days, with mean follow-up time at 1,364 days. Echocardiograms revealed the following: 12 (80%) patients showed either improved or stable left AV valve performance remaining at "mild" or less insufficiency, while two (13%) declined from "none" to mild and one (7%) from mild to "severe," which required reoperation. There was no residual shunting or LVOT obstruction at follow-up. The single (7%) reoperation was performed after three years due to left AV valve zone of apposition dehiscence. No permanent pacemakers were needed, and no deaths were reported. CONCLUSION: Our initial experience with CorMatrix in the repair of CAVSD in children has resulted in good initial and midterm outcomes. The CorMatrix patch remained stable through midterm follow-up, thus may be efficacious for use in CAVSD repair.

Increased regurgitant flow causes endocardial cushion defects in an avian embryonic model of congenital heart disease.

BACKGROUND: The relationship between changes in endocardial cushion and resultant congenital heart diseases (CHD) has yet to be established. It has been shown that increased regurgitant flow early in embryonic heart development leads to endocardial cushion defects, but it remains unclear how abnormal endocardial cushions during the looping stages might affect the fully septated heart. The goal of this study was to reproducibly alter blood flow in vivo and then quantify the resultant effects on morphology of endocardial cushions in the looping heart and on CHDs in the septated heart. METHODS: Optical pacing was applied to create regurgitant flow in embryonic hearts, and optical coherence tomography (OCT) was utilized to quantify regurgitation and morphology. Embryonic quail hearts were optically paced at 3 Hz (180 bpm, well above intrinsic rate 60-110 bpm) at stage 13 of development (3-4 weeks human) for 5 min. Pacing fatigued the heart and led to at least 1 h of increased regurgitant flow. Resultant morphological changes were quantified with OCT imaging at stage 19 (cardiac looping-4-5 weeks human) or stage 35 (4 chambered heart-8 weeks human). RESULTS: All paced embryos imaged at stage 19 displayed structural changes in cardiac cushions. The amount of regurgitant flow immediately after pacing was inversely correlated with cardiac cushion size 24-h post pacing (P value < .01). The embryos with the most regurgitant flow and smallest cushions after pacing had a decreased survival rate at 8 days (P < .05), indicating that those most severe endocardial cushion defects were lethal. Of the embryos that survived to stage 35, 17/18 exhibited CHDs including valve defects, ventricular septal defects, hypoplastic ventricles, and common AV canal. CONCLUSION: The data illustrate a strong inverse relationship in which regurgitant flow precedes abnormal and smaller cardiac cushions, resulting in the development of CHDs.

Bi-Luminal Mitral Valve: Incidence, Clinical Features, Associated Anomaly and Echocardiographic Evaluation.

OBJECTIVE: The aim of the study was to know the incidence, clinical features, associated anomaly and echocardiographic evaluation of bi-luminal mitral valve (also known as double orifice mitral valve or DOMV) in patients with suspected mitral valve disease, continous murmur or left-to-right shunt. METHODS: Twenty-eight patients with DOMV were diagnosed by transthoracic echocardiography (TTE) in a retrospective review of 52,256 echocardiographic studies in 45,898 patients performed between 2000 and 2015. RESULTS: The mean age was 20.1 years (15 - 34 years) with female preponderance (M/F: 1:1.8). Dyspnea and diastolic murmur were the most common symptoms found in 19 (67.8%) and 19 (67.8%) of patients, respectively. Normal sinus rhythm was the most common electrocardiographic finding. Twenty-five (89%) patients had complete bridge, while three (11%) had incomplete bridge type of DOMV. Twenty-one (75%) had severe mitral stenosis (MS) including severe tricuspid regurgitation (n = 13, 61%), ventricular septal defect (VSD, n = 3, 14%), complete endocardial cushion defect (ECD, n = 3, 14%), and mild to moderate mitral regurgitation (MR) (n = 2, 11%), moderate MS and moderate MR were found in four (16%) patients among complete bridge type of DOMV, while all patients with incomplete bridge type had severe MS and patent ductus arteriosus (PDA) as associated lesions. Overall, 24 (85%) had severe and four (15%) had moderate MS. CONCLUSIONS: DOMV as a cause of symptomatic mitral valve disease was seen in young and middle-aged patients with estimated incidence of 0.06%. Dyspnea and diastolic murmur were the most common symptoms. Mostly, it was an isolated anomaly but in majority, associated with VSD, complete ECD and PDA. TTE examination is a reliable and sufficient means of diagnosing DOMV and determining its type.

Atrioventricular septal defect: From embryonic development to long-term follow-up.

Atrioventricular septal defect (AVSD) covers a spectrum of heart anomalies with a common atrioventricular connection and has an incidence of 4-5.3 per 10.000 live births. About half of the AVSDs occur in patient with Down syndrome. This review provides a bench to bedside overview of AVSD. Developmental aspects, nomenclature, anatomy, and classification of AVSD are discussed. Furthermore an overview of genetic and maternal risk factors for AVSD is provided, and available literature on (fetal) diagnosis, surgical techniques and follow-up is presented. Special attention is given to differences in developmental, anatomical and prognostic factors of AVSD between non-syndromic and Down syndrome patients.

Relationship-Based Care for Newborns With Down Syndrome and Endocardial Cushion Defect.

Down syndrome with endocardial cushion defect is a challenging diagnosis for parents as well as members of the health care team. Utilizing a framework of relationship-based care, nurses are in a position to positively affect parents' experience by providing education, advocacy, and support from initial diagnosis through discharge. The plan of care is multidisciplinary and focuses on critical developmental needs, such as bonding and feeding. Because Down syndrome is associated with multiple anomalies, anticipatory guidance is needed to assist parents with establishing a health maintenance plan for their child after discharge.

Long-term outcomes after surgical repair of complete atrioventricular septal defect.

OBJECTIVE: Survival after surgical repair for complete atrioventricular septal defect (CAVSD) has improved, but patients are at risk for reoperation to address left atrioventricular valve regurgitation and left ventricular outflow tract obstruction. We examined the long-term survival, need for reoperation, and associated risk factors after CAVSD repair at our institution. METHODS: Between 1974 and 2000, a total of 198 patients underwent surgical repair for CAVSD. Of these, 178 patients survived to hospital discharge, of whom 153 (86%) had available follow-up data at a median postoperative time point of 17.2 years (range: 2 months to 38.1 years). RESULTS: Overall perioperative mortality was 10.1%, with a significant decrease to 2.9% in the late surgical era: 1991 to 2000 (P < .001). The overall estimated survival for the entire cohort was 85% at 10 years, 82% at 20 years, and 71% at 30 years after initial CAVSD repair. Requiring a reoperation after initial CAVSD repair was a risk factor for late mortality (P = .04). The estimated freedom from reoperation was 88% at 10 years, 83% at 20 years, and 78% at 30 years after initial CAVSD repair. Indications for reoperation included left atrioventricular valve regurgitation in 14 patients (7.1%) and left ventricular outflow obstruction in 7 patients (3.5%). CONCLUSIONS: Long-term survival after repair of CAVSD remains good. However, the need for reoperation is common and affects long-term survival after CAVSD repair.

Severe Left Atrioventricular Valve Regurgitation Due to Discontinuity between the Leaflets of the Aortic and Left Atrioventricular Valves in a Patient with Endocardial Cushion Defect: A Rare Case Report.

Discontinuities between the leaflets of the aortic and left atrioventricular valves are uncommon congenital malformations. The anomaly may be discovered during surgery without preoperative diagnosis. It represents a spectrum of anomalies that result from interruption of the normal development of the endocardial cushions during the fetal life. We describe a rare case of Down syndrome with transient complete atrioventricular block and discontinuity between the leaflets of the aortic and left atrioventricular valves without intervening fibrous band, leading to separation and detachment between them. It caused severe eccentric jet of regurgitation originated from left ventricular outflow tract and base of anterior leaflet of left atrioventricular valve into the left atrium. He underwent cardiopulmonary bypass, and the defect between left atrioventricular valve and aortic annuli was sewn. Permanent epicardial pacing was inserted during cardiac surgery. To the best of our knowledge, such a case has not been previously reported in the literature.