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British Heart Journal Jul 1953
Topics: Humans; Tricuspid Atresia; Tricuspid Valve
PubMed: 13059216
DOI: 10.1136/hrt.15.3.287 -
Journal of the American College of... Sep 1995Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in...
OBJECTIVES
Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in two-dimensional echocardiograms.
BACKGROUND
Traditionally, tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle.
METHODS
We examined 39 autopsy specimens catalogued as having tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of tricuspid atresia.
RESULTS
Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass.
CONCLUSIONS
Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.
Topics: Echocardiography; Heart Septal Defects, Ventricular; Heart Septum; Heart Ventricles; Humans; Infant, Newborn; Pennsylvania; Retrospective Studies; Tricuspid Atresia; Tricuspid Valve
PubMed: 7642870
DOI: 10.1016/0735-1097(95)00250-8 -
Journal of Equine Science Dec 2023The necropsy of a 2-day-old Noma horse that died of weakness showed an enlarged cardiac base and a narrow cardiac apex, suggesting cardiac malformation. The excised...
Postmortem magnetic resonance imaging findings of tricuspid atresia with ventricular and atrial septal defects and subvalvular pulmonic stenosis in a Japanese native Noma horse.
The necropsy of a 2-day-old Noma horse that died of weakness showed an enlarged cardiac base and a narrow cardiac apex, suggesting cardiac malformation. The excised heart underwent imaging to investigate its luminal structure. On three-dimensional magnetic resonance imaging, the right atrium and right ventricle were discontinuous. The right atrium communicated with the left atrium and the left ventricle communicated with the right ventricle. The lumen narrowed near the pulmonary artery valve. Since the same findings were observed on gross examination, the foal was diagnosed with tricuspid atresia with ventricular and atrial septal defects, along with subvalvular pulmonic stenosis.
PubMed: 38274557
DOI: 10.1294/jes.34.121 -
BMC Cardiovascular Disorders Mar 2022To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS).
BACKGROUND
To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS).
METHODS
50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled. Children with Type I were divided into groups A (n = 25) and B (n = 25). Group A had received BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, whereas group B had undergone BT shunt combined with PDA ligation and pulmonary valve incision. Children with type II were divided into groups C (n = 25) and D (n = 25). Group C had received BT shunt combined with PDA ligation, right ventricular outflow tract (RVOT) incision and transannular patch. Group D had undergone BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation. The differences in mechanical ventilation time, length of ICU stay, mortality rate, tricuspid Z value, tricuspid regurgitation, oxygen saturation, pulmonary regurgitation, McGoon ratio, pulmonary artery transvalvular pressure, survival rate were compared between groups A and B, between groups C and D respectively.
RESULTS
The ventilator assistance time and length of ICU stay were greater in group C than in group D (80.96 ± 8.42 h vs. 65.16 ± 4.85 h, P = 0.045; 222.00 ± 11.72 h vs. 162.48 ± 7.91 h, P = 0.048). The pulmonary artery transvalvular pressure was significantly higher in group A than in group B at 3, 6, 12, 24 and 36 months after surgery (64.86 ± 4.13 mmHg vs. 53.04 ± 5.64 mmHg, P = 0.045; 69.47 ± 1.93 mmHg vs. 55.95 ± 4.04 mmHg, P = 0.005; 80.16 ± 3.76 mmHg vs. 73.24 ± 2.34 mmHg, P = 0.035; 62.95 ± 5.64 mmHg vs. 48.47 ± 7.44 mmHg, P = 0.04; 53.69 ± 4.89 vs. 45.77 ± 3.26, P = 0.02). Furthermore, the tricuspid Z value was significantly greater in group B than in group A at 3 and 24 months after surgery (- (1.37 ± 0.04) vs. - (1.43 ± 0.06), P = 0.03; - (0.41 ± 0.06) vs. - (0.51 ± 0.11), P = 0.02).
CONCLUSIONS
The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and the effect of BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch.
Topics: Child; Heart Defects, Congenital; Humans; Infant; Pulmonary Atresia; Pulmonary Valve; Pulmonary Valve Insufficiency; Treatment Outcome
PubMed: 35300595
DOI: 10.1186/s12872-022-02549-1 -
Iranian Journal of Medical Sciences Jul 2017Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial...
Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. Skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. They range from clinodactyly, absent or digitalised thumb, hypoplastic or absent radii, and first metacarpal to hypoplastic ulna and carpal bone anomalies. Cardiac involvement ranges from asymptomatic conduction disturbances to multiple structural defects. Structural defects are seen in 75% of the cases and include both atrial and ventricular septal defect. More complex cardiac lesions such as Tetrology of Fallot, endocardial cushion defects, double outlet right ventricle, and total anomalous pulmonary venous return are observed uncommonly. An aneurysm of the interatrium septum is an infrequent finding in infants. It has been speculated that atrial septal aneurysm (ASA) is a direct source of thrombus formation. Paradoxical embolism of venous thrombi across a right to left shunt is possibly responsible for the cryptogenic stroke in a patient with ASA. However, coagulopathy associated with cyanotic congenital heart defect may also be contributory. Our patient had a rare association of complex cardiac lesion (tricuspid atresia, pulmonary stenosis, atrial septal aneurysm) with cardiac conductive defects and left parietal infarct along with the usual skeletal abnormalities.
PubMed: 28761211
DOI: No ID Found -
Journal of the American College of... Jan 1991The echocardiographic studies and clinical course of 27 fetuses (mean gestational age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant...
The echocardiographic studies and clinical course of 27 fetuses (mean gestational age 26.9 weeks) diagnosed in utero with tricuspid valve disease and significant tricuspid regurgitation were reviewed. The diagnosis of Ebstein's anomaly was made in 17 of the fetuses, 7 had tricuspid valve dysplasia with poorly developed but normally attached leaflets and 2 had an unguarded tricuspid valve orifice with little or no identifiable tricuspid tissue. One fetus was excluded from data analysis because a more complex heart lesion was documented at autopsy. All fetuses had massive right atrial dilation and most who were serially studied had progressive right-sided cardiomegaly. Hydrops fetalis was found in six cases and atrial flutter in five. Associated cardiac lesions included pulmonary stenosis in five cases and pulmonary atresia in six. Four fetuses with normal forward pulmonary artery flow at the initial examination were found at subsequent study to have retrograde pulmonary artery and ductal flow in association with the development of pulmonary stenosis (n = 1) and pulmonary atresia (n = 3). On review of the clinical course of the 23 fetuses (excluding 3 with elective abortion), 48% of the fetuses died in utero and 35% who were liveborn died despite vigorous medical and, when necessary, surgical management, many of whom had severe congestive heart failure. Of the four infants who survived the neonatal period, three had a benign neonatal course, all of whom were diagnosed with mild to moderate Ebstein's anomaly; only one had pulmonary outflow obstruction. An additional finding at autopsy was significant lung hypoplasia documented in 10 of 19 autopsy reports.(ABSTRACT TRUNCATED AT 250 WORDS)
Topics: Ebstein Anomaly; Echocardiography; Female; Fetal Diseases; Fetal Heart; Humans; Pregnancy; Retrospective Studies; Tricuspid Valve; Tricuspid Valve Insufficiency; Ultrasonography, Prenatal
PubMed: 1987222
DOI: 10.1016/0735-1097(91)90722-l -
Journal of the American College of... Mar 1991The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are...
The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are presented in detail. Twelve cases were documented by postmortem examination and the diagnosis was confirmed by echocardiography and surgical observation in the one living patient. Clinical data available in nine cases and cardiac catheterization data obtained in eight are included in this report. In three cases (23%)--two with tricuspid atresia and one with extreme tricuspid stenosis--the tricuspid valve and right ventricle exhibited characteristics seen in Ebstein's anomaly. In all 13 cases, the great arteries were normally related. The ventricular septal defect(s) in 10 (83%) of the 12 postmortem cases rapidly became smaller and this resulted in marked diminution of the pulmonary blood flow and severe hypoxia. Only three of the eight patients with available cardiac catheterization and angiocardiographic data showed the scooped-out appearance of the left ventricular septal surface characteristic of AV canal defects. By contrast, two-dimensional echocardiography, available in the three most recent cases, accurately demonstrated all the defects present and represents the diagnostic method of choice. Early surgical intervention to establish a systemic to pulmonary artery anastomosis is essential for survival. More definitive surgical treatment can be achieved later by an atriopulmonary or cavopulmonary anastomosis with or without replacement of the cleft and often regurgitant mitral valve. The one living patient exemplifies this approach. This is the largest series of this unusual type of tricuspid atresia reported to date.
Topics: Cardiac Catheterization; Ebstein Anomaly; Echocardiography; Electrocardiography; Endocardial Cushion Defects; Female; Humans; Infant; Male; Myocardium; Tricuspid Valve; Tricuspid Valve Stenosis
PubMed: 1999631
DOI: 10.1016/0735-1097(91)90876-b -
AJP Reports Oct 2013We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features...
We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of isomerism of the right atrial appendages has not previously been diagnosed or reported prenatally. Because of the complexity of cardiac lesions that may be present in cases of atrial isomerism, these disorders should be considered even if sonographic findings are uncommon or atypical.
PubMed: 24147244
DOI: 10.1055/s-0033-1344004 -
Frontiers in Pediatrics 2021Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to... (Review)
Review
Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe-and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. The mechanisms underlying improvements in aerobic exercise capacity and the effects of exercise training on circulatory and end-organ function remain incompletely understood. Furthermore, the optimal methods of exercise prescription are poorly characterized. This highlights the need for large, well-designed, multi-center, randomized, controlled trials. The Fontan Fitness Intervention Trial (F-FIT)-a phase III clinical trial-aims to optimize exercise prescription and delivery in people with a Fontan circulation. In this multi-center, randomized, controlled study, eligible Fontan participants will be randomized to either a 4-month supervised aerobic and resistance exercise training program of moderate-to-vigorous intensity followed by an 8-month maintenance phase; or usual care (control group). Adolescent and adult (≥16 years) Fontan participants will be randomized to either traditional face-to-face exercise training, telehealth exercise training, or usual care in a three-arm trial with an allocation of 2:2:1 (traditional:telehealth:control). Children (<16 years) will be randomized to either a physical activity and exercise program of moderate-to-vigorous intensity or usual care in a two-arm trial with a 1:1 allocation. The primary outcome is a change in aerobic exercise capacity (peak oxygen uptake) at 4-months. Secondary outcomes include safety, and changes in cardiopulmonary exercise testing measures, peripheral venous pressure, respiratory muscle and lung function, body composition, liver stiffness, neuropsychological and neurocognitive function, physical activity levels, dietary and nutritional status, vascular function, neurohormonal activation, metabolites, cardiac function, quality of life, musculoskeletal fitness, and health care utilization. Outcome measures will be assessed at baseline, 4-months, and 12-months. This manuscript will describe the pathophysiology of exercise intolerance in the Fontan circulation and the rationale and protocol for the F-FIT.
PubMed: 35071139
DOI: 10.3389/fped.2021.799125 -
European Heart Journal. Case Reports Apr 2023d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare...
BACKGROUND
d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma.
CASE SUMMARY
A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation.
DISCUSSION
A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient's unique cardiac haemodynamics and after shared decision-making with the patient.
PubMed: 37090763
DOI: 10.1093/ehjcr/ytad154