Serum High Sensitive Cardiac Troponin-T (hs-cTnT) as a Biomarker in Pediatric Pulmonary Hypertension due to Congenital Left to Right Cardiac Shunt

Background: Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD) with left-to-right shunt. Increased pulmonary pressure leads to vascular remodeling and RV dysfunction. Objectives: To analyze the role of high-sensitive cardiac troponin T (hs -cTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) in children with left to right cardiac shunt. Patients and Methods: Twenty patients with congenital heart disease (CHD) with left to right shunt and PAH-CHD, 20 patients with CHD with left to right shunt but without PH, and 20 healthy children, in total 60 individuals, were included in the study. All cases aged between 3 and 36 months. Plain x-ray chest and heart, electrocardiography, Doppler and Two –dimensional, M- mode echocardiographic evaluation of CHD and pulmonary pressure were performed in all patients. Blood samples were obtained from all cases for measurement of serum high-sensitive cardiac troponin T (hs-cTnT) levels by highly sensitive third-generation quantitative test. Results: The mean hs-cTnT levels were significantly higher in patients with PH than in patients without PH (p < 0.05) with a sensitivity 70% and specificity 95%. A statistically significant positive correlation was determined between pulmonary artery pressure and hs-cTnT levels, and significant negative correlation with EF% and FS %( by echocardiography). Conclusions: Serum levels of high-sensitive cardiac troponin T (hs-cTnT) were significantly elevated in PAH-CHD children denoting myocardial injury caused by volume and pressure load due to PH in children with left to right cardiac shunts, so it could be used as a cardiac biomarker in PAH-CHD children with good diagnostic and prognostic value and high sensitivity and specificity, which may be useful in the management of PH in childhood.

1249 Reverse platypnoea-orthodeoxia syndrome: A rare cause of hypoxemia during in lab polysomnography

Introduction Sleep related hypoxemia carries a broad differential diagnosis. Right-to-left shunting is a known cause of hypoxemia that is not correctable with supplemental oxygen. A patent foramen ovale (PFO) is an intra-cardiac shunt that results in hypoxemia and in rare instances can lead to platypnea-orthodeoxia syndrome (POS). POS is characterized by dyspnea and hypoxemia in the upright position, which improves when supine. We present a case of nocturnal hypoxemia and PFO with a unique clinical presentation consistent with reverse POS discovered on PSG. Report of Case 57-year-old male with Class I obesity and a remote diagnosis of mild sleep-disordered breathing not treated with CPAP was referred to Sleep Medicine for excessive daytime sleepiness, snoring, and witnessed apneas. Polysomnography revealed basal oxygen saturation of ~88% during sleep. There was no evidence of apnea or hypopnea. Hypoxemia was not correctable with supplemental oxygen, suggestive of shunt physiology. Oxygen saturation was normal during upright exercise. Pulmonary hypertension was ruled out. Transesophageal echocardiogram revealed PFO with right-to-left shunt. Overall presentation was consistent with reverse POS secondary to PFO, which will be treated with percutaneous trans-catheter closure. Conclusion This is a rare case of reverse POS secondary to PFO, with shunt physiology initially suspected during Sleep Medicine evaluation based on PSG. Reverse POS is characterized by dyspnea and hypoxemia while supine, as opposed to upright as in classic POS. The precise pathophysiology of reverse POS is unclear. This case emphasizes the need to consider reverse POS in patients with supine hypoxemia refractory to oxygen therapy. Such findings on PSG should prompt further workup for causes of right-to-left shunt. This diagnosis should not be overlooked, as the underlying abnormality is often correctable. In this patient, we expect hypoxemia to resolve with percutaneous trans-catheter closure of PFO.

Platypnea-orthodeoxia syndrome after open prostatectomy and cystolithotomy: Coincidence or unknown pathophysiology?

A few cases of platypnea-orthodeoxia syndrome have been described in the literature, some of them after thoracic or upper abdominal surgeries. In most cases, hypoxemia in the upright or sitting position, which is the main clinical symptom for this uncommon diagnosis, is usually related to a dynamic right to left cardiac shunt induced by anatomical changes in the relative position between the inferior vena cava and the atria in the presence of a patent foramen ovale. In this case report, we describe a situation in which platypnea-orthodeoxia syndrome developed acutely before surgery but that became severely exacerbated after an open urologic surgery without a clear acute anatomical change that could be responsible for triggering the syndrome. This case might suggest that the pathophysiology of acute platypnea-orthodeoxia syndrome is not completely elucidated and that other possible triggers for acute clinical manifestation in addition to acute anatomical thoracic changes must be explored.

P1729 Abnomral course of pulmonary vein causing right heart dilatation

Funding Acknowledgements None Introduction Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. It is accounted for 0.7%. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We report a case of a young female, incidentally found to have a right upper pulmonary vein draining into the right atrium, confirmed by TOE and CT Venography pulmonary, with moderate right ventricular enlargement, without pulmonary hypertension, intra-cardiac shunt or accompanied congenital anomaly. A decision of surgical correction was made. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, even in absence of ASD. Case presentation A 26-year-old female, not known to have any chronic illness. She was referred because of poor appetite and low weight, to cardiology evaluation for accidental murmur finding during examination. She had no other symptoms. The TOE was done which showed left pulmonary veins are normal, the right lower pulmonary vein is normal, and there is high suspicion of aberrant right upper pulmonary vein draining in the right atrium, no evidence of intra-cardiac shunt. CT Venography pulmonary revealed anomalous (Giant) right superior pulmonary vein to the superior vena cava and draining to the right atrium. Figure 1. Patient was referred for surgical correction. Discussion Partial anomalous pulmonary venous return is a type of left to right shunt. It is a rare congenital abnormal cardiac defect in which the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Its accounted for 0.4 to 0.7%. However, as this rate is based upon autopsy data, the true prevalence of PAPVR may actually be higher. PAPVR is frequently associated with other congenital heart disease, most commonly ASD, in &gt;75% of patients. It is estimated that 10% to 15% of patients with ASD and up to 85% of patients with sinus venosus ASD have PAPVC. Exclusive of ASD, other more complex cardiac malformations occur in ∼20% of patients. PAPVR can also be an isolated defect with intact atrial septum. TOE is more sensitive than TTE in detecting PAPVR. A potential limitation of echocardiography is the availability of acoustic windows. If all four PVs are not identified emptying into the left atrium on TEE, CCT and CMR may provide complementary data . CONCLUSION In conclusion, missed diagnosis always occurs in PAPVR, the debauched and blood flow of pulmonary vein should be over-viewed carefully during TEE examination. In a patient with unexplained RV enlargement PAPVC (one or more pulmonary veins) should be considered in the differential diagnosis. Multi-modality cardiac imaging using echocardiography, CT angiography and CMR may provide a comprehensive noninvasive evaluation of PAPVC. Abstract P1729 Figure. Fig1 TOE (Top),CT (Bottom) of RUPV

Medical Therapy for Eisenmenger Syndrome: A Case Report and Review of Literature

Eisenmenger syndrome (ES) is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect is eventually reversed into a cyanotic right-to-left shunt. It is crucial to recognize this grave pathology at the earliest because once it develops, treatment by medical or surgical means becomes even more challenging. In past decades, various therapeutic options have been developed that address the specific pathophysiological aspects of the disease and have shown to improve functional capacity and quality of life. There are three major therapeutic pathways in pulmonary hypertension treatment - endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacyclin derivatives. These therapies not only improve hemodynamic parameters and exercise capacity but they also improve prognosis with various form of Pulmonary hypertension including ES. We report a case of a 35-year-old female of ostium secundum atrial septal defect with ES, started on upfront combination therapy of ambrisentan and tadalafil who demonstrated marked improvement after 3 months of medical therapy.

Valve disease

Although echocardiography remains the primary imaging modality for valvular heart disease (VHD) diagnosis, cardiac magnetic resonance (CMR) has gained much interest in this field over the last few years. CMR allows for three-dimensional imaging of the cardiovascular system, using a large field of view, and reconstruction in any given plane. Its capability to quantify flow allows for accurate measurement of regurgitation, cardiac shunt volumes/ratios, and differential flow volumes (e.g. left and right pulmonary arteries). In addition, CMR provides insights into the aetiology/mechanism of VHD, the precise quantification of VHD severity, and the evaluation of myocardial response (function, remodelling, and fibrosis). This chapter discusses several CMR techniques for evaluation of patients with VHD. Important tips and pitfalls in the image acquisition and post-processing analysis will be also discussed, providing the users the necessary framework for its clinical application.