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'Tricuspid Atresia' (1994)


Feb
2018

Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published.

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Feb
2018

This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects.

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Feb
2018

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy.

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Jan
2018

Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis.

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Mar
2018

Pulmonary artery wedge pressure (PAWP) has been shown to correlate better with left atrial pressure (LAP) than ventricular end-diastolic pressure (VEDP) in acquired heart disease. The correlation between VEDP and PAWP and their performance as surrogates for LAP in Fontan patients is unknown.
Offline single-beat simultaneous measurement of PAWP and VEDP was performed in 50 adult Fontan patients and non-simultaneous hemodynamic data abstracted for calculation of pulmonary vascular resistance (PVR).

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Dec
2017

Conjoined twins are uncommon with reported incidences of 1 in 30,000-200,000 births. They represent a heterogeneous population in regard to location of joint body parts and presence/extent of internal organ fusion. Positioning, airway management, possible presence of cross-circulation, and the fact that 2 patients require anesthesia for each procedure present significant challenges to the anesthesiologist.

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Dec
2017

The aim of the study was to assess indications, procedural success, complications, echocardiographic, and clinical outcomes of percutaneous pulmonary valve implantation (PPVI) in adult patients with congenital heart disease (CHD).
PPVI offers a non-surgical treatment option for failing prosthetic conduits in pulmonary position. However, efficacy and clinical outcomes after PPVI are still underreported.

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Dec
2017

Staged palliative surgery markedly shifts the balance of volume load on a single ventricle and pulmonary vascular bed. Blalock-Taussig shunt necessitates a single ventricle eject blood to both the systemic and pulmonary circulation. On the contrary, bidirectional cavopulmonary shunt release the single ventricle from pulmonary circulation.

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Feb
2018

A 4-week-old American Quarter Horse colt presented with a recent history of diarrhea and decreased activity level. On initial physical examination, the animal was bright and alert and major findings were limited to a loud systolic heart murmur radiating widely over both sides of the thorax. While in the hospital, the clinical condition of the foal warranted further imaging to determine the cause and extent of cardiac disease.

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Oct
2017

BACKGROUND To summarize our clinical experience in performing transthoracic balloon pulmonary valvuloplasty for the treatment of patients suffering from congenial pulmonary atresia with intact ventricular septum (PA/IVS). MATERIAL AND METHODS Between April 2009 and April 2016, 38 patients with PA/IVS underwent transthoracic balloon pulmonary valvuloplasty in our hospital. All of them were combined with patent ductus arteriosus, tricuspid insufficiency, and atrial septal defect or patent foramen ovale.

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Nov
2017

The life journey of an Indian pediatric cardiologist, who bestowed considerable attention to the development of new knowledge and train/teach physicians around the world while providing care of patients with heart disease over a 45-y period, is reviewed. This appraisal focuses particular attention on the scientific contributions to the literature. These include spontaneous closure of physiologically advantageous ventricular septal defects, various issues related to a congenital heart defect namely, tricuspid atresia and transcatheter and, interventional pediatric cardiac procedures.

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Sep
2017

There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.
All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%.

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Aug
2017

Pulmonary atresia with intact ventricular septum (PA-IVS) is an uncommon disorder with significant morphological heterogeneity. The use of percutaneous radiofrequency (RF)-assisted perforation of the atretic valve and subsequent balloon dilation provides a relatively easy but expensive procedure that is expected to establish ante-grade flow through the pulmonary valve in most patients.
The aim of the study was to attempt a cost reduction by using catheters and wires readily available in our catheter laboratory.

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Aug
2017

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status.

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Dec
2017

Fetuses with pulmonary atresia or pulmonary stenosis with intact ventricular septum manifest variable degrees of right ventricle hypoplasia and inadequacy. We studied the relationship between prenatal echocardiographic parameters and their progression through gestation as potential predictors of postnatal single-ventricle or two-ventricle care strategy. Serial fetal echocardiograms of pulmonary atresia (n = 28) or severe pulmonary stenosis (n = 8) and intact ventricular septum were reviewed.

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Jul
2017

Tricuspid atresia (TA) is a rare congenital heart defect in which the right atrioventricular connection, the tricuspid valve, is absent. As a result, there is no direct communication between the right atrium and right ventricle. Surgical treatment, including the Fontan procedure, is indicated yet palliative, leaving patients with various lifelong complications.

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Jul
2017

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.

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Jan
2018

Pulmonary atresia with intact ventricular septum is a unique congenital malformation of the heart in which patients may undergo single-, two- or 1½- ventricle repair. Size of the tricuspid valve annulus, morphology of the right ventricle and presence of ventriculocoronary connections can all impact the selection of appropriate palliative strategy. We developed the aortic perfusion score, a novel scoring system based on anterograde coronary perfusion with the aim of being able to identify patients at risk for death or transplant.

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Oct
2017

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.

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Jun
2017

Fetal dextrocardia is a type of cardiac malposition where the major axis from base to apex points to the right side. This condition is usually associated with a wide spectrum of complex cardiac defects. As a result, dextrocardia is conceptually difficult to understand and diagnose on prenatal ultrasound.

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Jul
2017

The cyanotic congenital heart diseases are a rare and heterogeneous group of disorders, often requiring urgent neonatal management. Although echocardiography is the mainstay for imaging, continued technological advances have expanded the role for computed tomography and magnetic resonance imaging, helping to limit invasive cardiac catheterization. In this article, the authors review the broad spectrum of cyanotic congenital heart disease, focusing on the utility of advanced noninvasive imaging modalities while highlighting key clinical features and management considerations.

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Aug
2017

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.

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Dec
1969

The Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors.

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Dec
1969

In the surgical treatment of pulmonary atresia with intact ventricular septum, the size of the tricuspid valve annulus (as measured by z-scores) has emerged as a significant factor in deciding which repair to perform. Various tricuspid valve annulus z-scores are reported as "cutoffs" for successful biventricular repair. We aimed to determine whether the use of different z-score data sets contributed to the gross variation in "cutoffs" for successful biventricular repair reported in the literature.

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Dec
1969

Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail.
The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk.

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May
2017

Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies.
Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers.

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Aug
2017

Patients undergoing palliative surgeries for single-ventricle conditions are affected by multiple comorbidities or non-cardiac conditions. The prevalence, costs and the cost implications of these conditions have not been assessed.
Administrative costing records from four hospitals in Australia and New Zealand were linked with the Fontan registry database to analyze the inpatient resource use for co-morbid or non-cardiac conditions.

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Apr
2017

Page kidney is an uncommon condition that hypertension occurs secondary to microvascular ischemia and alternation of small-vessel hemodynamics due to external compression of renal parenchyma and activation of the renin-angiotensin-aldosterone system. There are no specific guidelines for the management of Page kidney in the literatures.
A 17-year-old teenager who had Fontan procedure for tricuspid and pulmonary atresia in early childhood suffered from sudden onset of severe left flank pain during cardiac catheterization procedure.

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Jun
2017

In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes.

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May
2017

Genetic variation in specific transcription factors during heart formation may lead to congenital heart disease (CHD) or even miscarriage. The aim of the present study was to identify CHD‑associated genes using next generation sequencing (NGS). The whole exome DNA sequence was obtained from a stillborn fetus diagnosed with tricuspid atresia and complete transposition of the great arteries using high‑throughput sequencing methods.

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May
2017

We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach.
This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia. Initial results led to tightening of the pulmonary artery band from 3.

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Jun
2017

In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined.

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Apr
2017

Veno-venous collaterals are sometimes seen in patients after the Fontan procedure. A 28-year-old female with tricuspid atresia who underwent the Fontan procedure had oxygen desaturation due to a giant veno-venous collateral. Coil embolization was performed for the collateral.

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Mar
2017

Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age.

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Apr
2017

Most patients with single ventricle (SV) congenital heart disease are expected to survive to adulthood. Women with SV are often counseled against pregnancy; however, data on pregnancies in these women are lacking. We sought to evaluate in-hospital outcomes of pregnancy in women with SV.

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Apr
2017

Very few studies have been conducted in this part of world to identify relation between maternal serum homocysteine levels and congenital heart disease in their offsprings. With this perspective in mind, this study was carried out.
Fifty women were enrolled in this study.

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Jul
2017

The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings.
We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit.

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Dec
2016

Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal.

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Dec
2016

Congenital heart disease is associated with abnormal ventricular shape that can affect wall mechanics and may be predictive of long-term adverse outcomes. Atlas-based parametric shape analysis was used to analyze ventricular geometries of eight adolescent or adult single-ventricle CHD patients with tricuspid atresia and Fontans. These patients were compared with an "atlas" of non-congenital asymptomatic volunteers, resulting in a set of z-scores which quantify deviations from the control population distribution on a patient-by-patient basis.

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Jul
2017

Cardiac haemangiomas are exceedingly rare; however, they can cause significant haemodynamic impairment and disturbances in heart rhythm. Rarely, cardiac tumours may also coexist with congenital heart lesions. We present an extremely unusual case of a cardiac haemangioma in the setting of complex transposition of the great arteries that caused functional tricuspid atresia.

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Jul
2017

The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed.

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Jan
2017

The management of some subsets of patients with an unsuccessful balloon atrial septostomy (BAS) and poor general condition is typically challenging. Our novel technique involves off-pump atrial septectomy using laparoscopic forceps guided by intraoperative direct pericardial echocardiography. The technique was first used in a case of tricuspid atresia in a patient in preshock and was then used in 2 more patients with hypoplastic left heart syndrome (HLHS) (age, 10-116 days; body weight, 2.

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Mar
2017

Ectopia cordis (EC) is a rare congenital anomaly often associated with congenital heart disease (CHD). There is a lack of contemporary information on EC diagnosed prenatally. We sought to combine the experiences of two regional referral centers in order to evaluate current outcomes for EC.

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Apr
2017

An increasing number of patients with congenital heart disease are now surviving into adulthood. This has also led to the emergence of complications from the underlying congenital heart disease, related surgical interventions, and associated combordities. While the prevalence of particular arrhythmias with specific congenital heart disease has been previously described, a detailed analysis of all lesions and a large number of comorbidities has not been previously published.

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Mar
2017

Left ventricle non-compaction (LVNC) has worse outcomes when associated with congenital heart defects (CHD). The co-occurrence and outcomes of LVNC with tricuspid atresia (TA) are not well described. Our study aims to determine the prevalence of LVNC with functionally single ventricle due to TA, and to describe the early outcomes of surgical palliation.

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Nov
2016

We present a case of a fetal diagnosis of tricuspid atresia (TA). The pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born.

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Apr
2017

A newborn with tricuspid atresia and pulmonary atresia underwent ductal stenting. The aortic end of the ductus was not completely covered and was wide open; the baby was discharged on dual antiplatelets. The baby presented after a month with desaturation, and an angiogram showed extensive thrombus in the stent and the right pulmonary artery.

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Jan
2017

Percutaneous radiofrequency perforation (RFP) of the pulmonary valve is used as a primary therapy in neonates with pulmonary atresia and intact ventricular septum (PAIVS). We sought to determine the safety and efficacy of RFP for PAIVS in a single center and assess the pre-intervention anatomical parameters associated with a biventricular outcome. We retrospectively reviewed all cases of PAIVS treated with RFP at a single center from 1999 through 2012.

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