Objectives
A novel Occlutech atrial flow regulator (AFR) implantation gives an atrial septal predefined predictable fenestration.
Background
Atrial septostomy relieves syncope in pulmonary arterial ...hypertension (PAH) by improving left heart filling, cardiac output and systemic oxygen transport despite hypoxia. Symptoms recur when small fenestrations close spontaneously.
Methods
AFR was implanted after informed consent in patients with severe PAH presenting with syncope and right heart failure. Symptoms, hemodynamics, echocardiographic parameters, brain natriuretic peptide (BNP) levels and device patency were serially documented.
Results
Twelve patients aged 28.3 ± 8.5 years with severe PAH underwent AFR implantation. All procedures were successful without any major complications. All patients had relief of syncope and 6‐min walk distance improved significantly from 377.3 ± 33.2 to 423 ± 31.32 m. The cardiac index (2.36 ± 0.52 to 2.89 ± 0.56 L/min/m2) and systemic oxygen transport (367.5 ± 75.5 to 428.0 ± 67.1 ml/min/m2) also showed a significant improvement. Inferior caval vein congestion and pericardial effusion reduced due to improvement in heart failure, but other echocardiographic parameters of right ventricular function did not show significant change. The reduction in BNP levels too did not reach statistical significance. The device was patent in all patients at a median follow‐up of 189 days (range 10–296 days) resulting in a significant reduction of oxygen saturations from 98 ± 0.18 to 85.26 ± 2.86% after exercise.
Conclusions
AFR implantation was feasible and safe in all patients with PAH. There was a significant improvement of symptoms, six‐minute walk distance, cardiac index and systemic oxygen transport. The device maintained patency in short‐term follow‐up and the resultant hypoxia was tolerated well.
Background: The 'hepatic factor,' a molecule or group of molecules present in the hepatic
venous blood, essential for the prevention of the development of pulmonary arteriovenous malformations
...(PAVMs) and right-to-left shunting has been a conceptual enigma in the understanding of
many related conditions.
Methods: Patients with various forms of liver diseases including acute hepatic failure, and others
with normal hepatic function like hereditary hemorrhagic telangiectasia (HHT), inflammatory and
parasitic disorders, cardiogenic hepatopulmonary syndrome (cHPS) and skin disorders like Dyskeratosis
congenita are all known to cause PAVMs. Over a period of the last two decades our understanding
of the pathogenesis of PAVMs has changed, but the mechanisms are still not clearly understood.
The presence of PAVMs once considered a contraindication for liver transplantation is now a cure
for PAVMs in patients with HPS.
Results: In this article the molecular mechanisms and the underlying pathogenesis of PAVMs are
discussed and the role of microRNA (miRNA) in its pathogenesis is favorably argued. Identifying
and preventing or treating the underlying mechanisms will significantly influence the management of
a large group of patients who at present cannot be effectively treated with a very poor prognosis.
Progressive polycythemia, desaturation, stroke, and infection are serious complications of PAVMs.
Conclusion: The clinical data and current understanding leads to the possible role of miRNA, which
inhibits Vascular Endothelial Growth Factor (VEGF) synthesis as a pathogenic mechanism for the
development of PAVMs.
Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has ...been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.
Despite our growing experience in the medical care of extremely preterm infants and critically ill neonates, there are serious gaps in the understanding and clinical application of the adaptive ...physiology of the newborn. Neonatal physiology is often misinterpreted and considered similar to that of adult physiology. The human psyche has been seriously influenced, both from an evolutionary and survival point of view, by the cause and effect of hypoxemia which is considered as a warning sign of impending death. Within this context, it is unimaginable for even the highly trained professionals to consider saturation as low as 65% as acceptable. However, all available data suggests that newborns can thrive in a hypoxemic environment as they are conditioned to withstand extreme low saturations in the fetal environment. An approach utilizing the benefits of the hypoxic conditioning would prompt the practice of optimal targeted oxygen saturation range in the clinical management of the newborn. Our current understanding of cyanotic congenital heart disease and the physiology of single ventricle circulation, where oxygen saturation in mid 70s is acceptable, is supported by clinical and animal studies. This article argues the need to challenge our current acceptable target oxygen saturation in the newborn and provides the reasoning behind accepting lower target oxygen levels in the critically ill newborn. Challenging the current practice is expected to open a debate paving the way to understand the risks of high target oxygen levels in the newborn compared with the benefits of permissive hypoxia in avoiding the associated morbidity and mortality of oxygen radical injury.
Cardiac rhabdomyoma is the primary feature of the genetic disease, tuberous sclerosis complex, the most common cardiac tumor diagnosed in neonates and infants. Spontaneous regression is observed in ...most cases, but these tumors may cause hemodynamic instability, arrhythmias or other complications. We describe the case of a critically ill neonate, resuscitated after cardiac arrest secondary to massive locally invasive cardiac rhabdomyoma, who was successfully treated with everolimus (mammalian target of rapamycin mTOR inhibitor). Rapid tumor regression was observed on echocardiography, but it was unlikely that it was confounded by the natural disease course of regression. The presented case suggests that mTOR inhibitors may play a significant role in the treatment of large cardiac rhabdomyomas in critically ill neonates.
Background
Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and ...expanding the stent at the wider SVC‐RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10‐zig covered Cheatham‐platinum (CCP) stent has the advantage of being available in lengths of 5–11 cm and dilatable to 34 mm in diameter.
Methods
An international registry reviewed the outcomes of 10‐zig CCP stents in 75 patients aged 11.4–75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A “protective” balloon catheter was inflated in the RUPV in 17.
Results
Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p < .001) and RVEDVi from 149.1 ± 35.4 to 95.6 ± 21.43 ml/m2 (p < .001).
Conclusions
Ten‐zig CCPs of 7–8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.
Purpose of Review
Atrioventricular septal defects (AVSD) represent a broad spectrum of congenital anomalies from simple to the most complex heart defects including some distinct types. Clinical ...presentation and timing of intervention differ by morphological subset and functional anatomy. Herein, we review morphological variations and characteristics that determine appropriate intervention and provide insights into functional anatomy based on detailed three-dimensional (3D) assessment of AVSDs.
Recent Findings
The understanding of functional morphology of AVSDs has improved significantly with detailed 3D echocardiographic evaluation of the atrioventricular junction and valve morphology. As prenatal detection of AVSDs has increased significantly, it has become the most common fetal cardiac diagnosis enabling antenatal counseling and delivery planning. Advances in diagnosis and perioperative care have resulted in optimal outcomes.
Summary
The diagnosis and management of AVSDs have improved over the years with enhanced understanding of anatomy and perioperative care resulting in optimal short and long-term outcomes.
The ductus arteriosus is a muscular artery connecting two elastic arteries with different resistances. It is a normal fetal structure that only becomes pathological if it remains patent after birth. ...A varied clinical impact is observed as some neonates may be asymptomatic, symptoms may be deferred until later in life, or the infant may be overtly symptomatic and present as early as the first days of life. Prematurity increases the likelihood of persistent ductal patency and is seen in about 30% of preterm infants. In premature neonates, patent ductus arteriosus (PDA) is associated with significant morbidity and mortality 1,2. This discussion explains the pathophysiology behind the pathological events associated with PDA.
Increasingly end-organ injury is being demonstrated late after institution of the Fontan circulation, particularly liver fibrosis and cirrhosis. The exact mechanisms for these late phenomena remain ...largely elusive. Hypothesizing that exercise induces precipitous systemic venous hypertension and insufficient cardiac output for the exercise demand, that is, a possible mechanism for end-organ injury, we sought to demonstrate the dynamic exercise responses in systemic venous perfusion (SVP) and concurrent end-organ perfusion. Ten stable Fontan patients and 9 control subjects underwent incremental cycle ergometry–based cardiopulmonary exercise testing. SVP was monitored in the right upper limb, and regional tissue oxygen saturation was monitored in the brain and kidney using near-infrared spectroscopy. SVP rose profoundly in concert with workload in the Fontan group, described by the regression equation 15.97 + 0.073 watts per mm Hg. In contrast, SVP did not change in healthy controls. Regional renal (p <0.01) and cerebral tissue saturations (p <0.001) were significantly lower and decrease more rapidly in Fontan patients. We conclude that in a stable group of adult patients with Fontan circulation, high-intensity exercise was associated with systemic venous hypertension and reduced systemic oxygen delivery. This physiological substrate has the potential to contribute to end-organ injury.
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