A 16-year-old man with history of two weeks-flu like symptoms with intermittent fever. He came to the emergency department with 2 hours-chest pain that radiates to the back and upper extremities. At the admission he was hemodynamically stable with normal blood pressure The ECG showed sinus rhythm and ST segment elevation of 0.5 mV in all leads (Figure 1A). The cardiac enzymes were elevated (Troponin 12.19 ng/mLland creatine kinase-MB fraction 63.25 U/L). He was admitted to the Intensive Care Unit and later transferred to our medical unit to continue with study protocol. The transthoracic echocardiogram (Figure 1B) reported normal left ventricular systolic function with left ventricular ejection fraction (LVEF) 68%, global longitudinal strain -18%, TAPSE 30 mm, and normal systolic pulmonary artery pressure (30 mmHg).

Anomalies of coronary artery origin are congenital malformations characterized by the abnormal birth of a coronary artery from the controlateral coronary aortic sinus (birth of the left coronary artery from the right sinus or birth of the right coronary artery from the left sinus). The artery concerned has an abnormal initial path between the aorta and the pulmonary artery; this segment is most often intramural, in the aortic wall. They are rare with a prevalence (0.1% to 0.3% of the population). They pose a high risk of sudden stress death related to exercise myocardial ischemia. The most common mode of discovery is aborted sudden death, but sometimes fortuitously. The contribution of multi-round CT is described for the positive diagnosis of these anatomical variations, sometimes delicate in coronary angiography, but also for the distinction between “benign” and “malignant” forms, potentially responsible for myocardial ischemia. Treatment is usually surgical in symptomatic forms. We report the cases of two patients with coronary connection abnormalities discovered in adulthood.

A key platform underpinning the traditional understanding of the cardiovascular system, with respect to the behavior of large arterial vessels, is Otto Frank’s Windkessel Hypothesis [1]. This hypothesis posits simply that the smooth muscle walls of large arteries do not undergo rhythmic contractions in synchrony with the heartbeat but, rather, behave as passive elastic tubes undergoing distension from pulsatile pressure waves. The Windkessel Hypothesis is elegant, well described for over a century, ingrained in the understanding of cardiovascular medicine and physiology, and simply wrong. Several groups have now shown that the arterial smooth muscle wall undergoes rhythmic activation in synchrony with the heartbeat in a variety of tissues, including human brachial artery; canine coronary, femoral, and carotid arteries; rabbit aorta; feline pulmonary artery and rodent aorta [2-8]. The phasing of these events is such that the upstroke of the contraction slightly precedes the upstroke of the pulse wave, suggesting nomenclature for the events as pulse synchronized contractions, or PSCs [3,6-8]. PSCs have been found to be of neurogenic origin, sensitive to the neural blocker tetrodotoxin [3,8]. Although the specific neural pathways regulating PSCs have not been elucidated, the alpha-adrenergic system is at least partially involved, as evidenced by reduction or blockade of PSCs by the alpha-adrenergic blocker phentolamine [8]. Further, PSCs have not been observed following vessel excision in in vitro studies, as an intact nervous system is not present. The pacemaker for the PSC resides in the right atrium, as suggested by two lines of evidence. First, pacing of the right atrial region to faster than spontaneous frequencies leads to a one-to-one correspondence of PSC frequency with the stimulation rate [3]. Additionally, excision of the right, but not the left, atrial appendage results in elimination of PSCs [3]. As the pacemaker region for PSCs and the heartbeat both lie in the right atrium, this may potentially allow for coordination between the heartbeat and pulse wave with PSCs [3,5,8]. Extensive evaluations also have been performed showing the PSC was not an artifact produced either by cardiac contractility or from the vessel distension from the pulse wave [3,5,6].

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive disease without treatment that leads to death. Therefore, to control its progression to pulmonary hypertension is still a challenge. Moreover, there is no study that has investigated the Renin-Angiotensin System in patients with IPF. Objective: Verify the plasma concentrations of Angiotensin I, Angiotensin II (AngII), Angiotensin-(1-7) [Ang- (1-7)] and Alamandine in patients with IPF. Methods: Ten IPF patients, with or without PH, were included, and ten controls matched by sex and age. Quantitative plasma peptide concentrations (PPC) were expressed as mean and standard deviation or median and interquartile range. The Student Newman-Keuls t test was used for parametric data, Mann-Whitney for nonparametric data and, to compare proportions, the Fisher exact test was performed. The associations between clinical variables and the PPC were evaluated by Pearson or Spearman correlation coefficients. A p ≤ 0.05 was considered statistically significant. Results: The Alamandine plasma concentration was significantly (365%) lower in the IPF group and positively associated (r = 0.876) with pulmonary artery pressure (PAP). In addition, only in control group, the forced expiratory volume (FEV1%) was positively associated (p = 0.758) with Ang-(1-7). Conclusion: This study showed, for the first time, that there is a decrease in Alamandine participation in patients with IPF. The ACE-AngII-AT1 axis may be more active in this disease. In addition, our results suggest that Alamandine might be compensating the increase in PAP, as well as the Ang-(1-7) is improving the forced expiratory volume.

his patient (male, 59 years old) underwent cardiac re-transplantation for chronic rejection. Prior to re-transplantation, the patient was in NYHA class IV, with a clear chest x ray. On 14th postoperative day, he presented hemoptysis. On chest x-ray, a left lower lobe opacity was seen. Therefore, a chest CT scan was done and it showed a round mass within a pulmonary cavity surrounded by airspace in proximity of the pulmonary artery.

Introduction: Pulmonary hypertension (PH) is prevalent in hemodialysis (HD). In the general population, more women than men have PH due to left ventricular (LV) disease with preserved ejection fraction (EF). Little is known about the gender-specific prevalence of PH and associated LV abnormalities in patients with end stage renal disease (ESRD) on HD. Our aim was to evaluate gender differences and LV structural and functional changes in PH among ESRD patients on HD. Methods: Ninety-four patients (ages 23-77 years) underwent echocardiography after HD. Patients were divided based on estimated pulmonary artery systolic pressure (PASP) (Group A PASP < 40 mm Hg, Group B PASP ≥ 40 mm Hg). LV measurements included LV mass, LV internal dimensions, and LV ejection fraction (EF). LV diastolic function (LVDF) was assessed from mitral inflow deceleration time (DT) and E/A ratio. Results: Fifty-five patients (59%) had PH, including 32 of 49 men (65%) and 23 of 45 women (51%). LVEF was lower in Group B (46.4 ± 17.6 vs. 62.4 ± 14.4%, p < 0.001). Men with PH had higher LVIDd, cm (5.52 ± 0.89 vs 4.78 ± 0.75, p < 0.001), LVIDs, cm (3.75 ± 0.94 vs 3.14 ± 0.91, p = 0.03) LV mass, g (236 ± 74vs 189 ± 56, p = 0.02) and lower LVEF (40.0 ± 16.7 vs 52.0 ± 15.6, p = 0.008) than women. Conclusion: Patients on HD have a high prevalence of PH. PH was not associated with clear LV structural changes. There was a depression in LV systolic function without changes in LVDF. PH patients were more often men with hypertrophied LV with depressed LV systolic function. 

Background: Macitentan significantly improves pulmonary hemodynamics and survival in patients with primary pulmonary hypertension (PPH). Its beneficial effect, however, may be blunted due to the adverse impacts such as anemia and peripheral edema. Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity are associated with increased morbidity and mortality. We tried to evaluate that the effectiveness of the macitentan in patients with late-onset pulmonary hypertension after atrial septal defect operation in our center. Methods: The effect of a single dose of macitentan (10 mg) on pulmonary hemodynamics, functional capacity was examined in four patients with late-onset pulmonary hypertension after atrial septal defect operation. Results: The macitentan significantly improved mean pulmonary artery pressure (MPAP), cardiac output (CO), tricuspid annular plane systolic excursion (TAPSE), right ventricle systolic wave(RVS’), 6-minute walking test and NT-proBNP levels compared with before treatment. Conclusions: Macitentan can be used in patients with late-onset pulmonary hypertension after shunt operation especially atrial septal defect.

We report a case of pulmonary embolism complicated with pulmonary tuberculosis. A 48-year-old woman suffered from pulmonary tuberculosis more than 6 years without formal treatment. Recently, she went to hospital because of “chest tightness and dyspnea”, and died in the process of admission to hospital. Pulmonary embolism was found by autopsy and histological examination. We analyzed the relationship between pulmonary tuberculosis and pulmonary thromboembolism and the problems we should pay attention to in forensic pathology.

Background: Pulmonary artery bleeding secondary to iatrogenic injury is a troublesome intraoperative complication. The likelihood of encountering this complication is significantly higher in redo surgery for a number of reasons, including distortion of anatomical structures, adhesions and loss of tissue planes. Significant blood loss, although rare, remains a concern, and can occasionally be life-threatening. When significant bleeding from the pulmonary artery occurs, it can be a challenging situation to manage. Case Report: A 65 year old female was undergoing redo thoracic surgery in the setting of a completion lobectomy for biopsy-proven primary adenocarcinoma of the lung. Iatrogenic injury to the pulmonary artery resulted in significant bleeding that could not be managed by gaining proximal control due to dense adhesions. The novel decision to utilize a Foley catheter for balloon tamponade was taken, in order to provide sufficient haemostasis for definitive surgical repair of the defect to be undertaken Conclusion: The increased technical difficulties of redo thoracic surgery are well recognised. We describe the first case of Foley catheter balloon tamponade being utilized in the context of iatrogenic pulmonary artery bleeding during thoracic surgery.

Background: Down syndrome (DS), or Trisomy 21, is the most common genetic disorder in the world and congenital heart disease (CHD) contributes significantly to morbidity and mortality in this population. Early diagnosis and prompt cardiac intervention improve their quality of life. This study was done to determine the prevalence and pattern of congenital heart disease among children with Down syndrome seen at the Paediatric Cardiology Unit of Federal Medical Centre (FMC), Bayelsa State.Method: A prospective study of children with Down syndrome referred for cardiac evaluation and echocardiography at the Paediatric Cardiology Unit of FMC, Bayelsa State over four years from 1st January 2016 to 30th December 2019. Data on socio-demographic information, echocardiographic diagnosis, and outcome were retrieved from the study proforma and analyzed.Results: A total of 24 children with Down syndrome were seen over the study period. Their age ranged from 0 to 16years. The majority, 20 (83.3%) of the children with Down syndrome were aged 5 years and below. There were 13 males and 11 females with a male to female ratio of 1.2:1. A total of 23 (95.8%) of the children with Down syndrome had CHD. The most common CHD was AVSD (including complete, partial, isolated, or in association with other defects) in 66.6% followed by TOF in 8.3%. Multiple CHDs were seen in 43.5% of the children. Only one child (4.2%) had a structurally normal heart on echocardiography. All the children with Down syndrome had pericardial effusion of varying severity while 33% had pulmonary artery hypertension (PAH). The fatality rate among the children seen with Down syndrome over the study period was 34.8% and only one child (4.2%) had open-heart surgery with the total repair of cardiac defect during the study period. Conclusion: Morbidity and mortality are high among children with Down syndrome due to the high prevalence of CHD. Early referral, diagnosis, and prompt intervention are encouraged.

Chronic thromboembolic pulmonary hypertension is a notoriously underdiagnosed cause of severe pulmonary hypertension. It is a form of precapillary pulmonary hypertension (PH) that results from intraluminal thrombus organization and fibrous formation which ultimately results in the complete obliteration of pulmonary arteries, resulting in increased pulmonary vascular resistance which leads to the development of pulmonary hypertension and as a result right heart failure. The mechanism involves the narrowing of the pulmonary artery which increases blood pressure within the lungs and impairs blood flow which increases the workload of the right side of the heart ultimately causing right heart failure. Pulmonary hypertension can also cause arrhythmias, blood clots, and bleeding in the lungs. Even though CTEPH is a deadly condition, among all forms of pulmonary hypertension, CTEPH is the only curable form. Echocardiography is the initial assessment tool for suspected PH. A right heart catheterization may be performed to confirm the presence of pulmonary hypertension. Confirmation of CTEPH requires a V/Q scan. Although ventilation/perfusion scintigraphy has a major role in the evaluation of patients with suspected CTEPH, nowadays CTA chest is being used widely as it produces much better-quality images compared to V/Q scan. Without treatment, the prognosis is very poor. Out of three treatment modalities such as; pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), and medical therapy, surgery is the gold standard. The physician must be familiar with the disease entity, early diagnosis, and appropriate treatment to improve survival. Here we present a literature review on this topic.

Between coronary artery anomalies, myocardial bridging means an epicardial coronary artery, mostly left anterior descending artery (LAD), running through an intramyocardial “tunnel” (usually in the middle segment), leading during systolic contraction, flow reduction, through the vessel. When this anomaly is associated with a coronary fistula, which “steals” more from the bloodstream, the symptoms are more pronounced, and the management complex is surgical in particular. Despite the presence from birth remains asymptomatic and it becomes clinically manifest later in the third to fourth decade of life, with a diverse palette of symptoms; angina, arrhythmias, and acute myocardial infarction up to sudden death. Diagnosis and particular management, medical, interventional, and surgical should avoid major cardiac complications and sudden death. We present two adult patients, with coronary artery bridging, one case associated with coronary artery fistula, LAD to pulmonary artery trunk, very symptomatic with surgical management, and the second only myocardial bridging controlled with medication and supervision.

Pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with a mortality rate of 37%. There is an upregulation of adhesion molecules which leads to the expression of endothelin-1, a potent vasoconstrictor. A prospective, descriptive study was done to determine the safety and efficacy of macitentan in patients with SCD and PH. Continuous variables were reported as mean ± SEM or percentage where appropriate. We screened 13 patients and recruited five. All five patients were adults. Data were analyzed as appropriate by student t - test. Statistical significance was assumed at p < 0.05. Baseline pulmonary hemodynamics obtained by right heart catheterization and systemic hemodynamics were (± SEM): mean pulmonary artery pressure (MPAP) 32 ± 8 mmHg, right atrial pressure (RAP) 9 ± 4 mmHg, pulmonary vascular resistance (PVR) 257 dynes-sec/cm5 and CI 3·7 ± 0.39 l/m2. Of all parameters, only PVR and 6-min walk distance changed significantly. For the group, MPAP decreased by 15.6%, PVR by 22.5% and RAP by 25.5%. The 6-minute walk distance increased over sixteen weeks except in Patient 4 who had a 3% decrease. The mean walk distance increased in the total distance, from 464 ± 158 meters to 477 ± 190 meters (p .123). In four patients, the adverse events were mild to moderate and did not lead to study drug discontinuation. Significant improvement in pulmonary hemodynamics and exercise capacity in patients with SCD-related pulmonary arterial hypertension. We found that macitentan was safe and well tolerated.