Severe Pulmonary Artery Research Articles

Unusual pulmonary thromboembolism in a Dirofilaria immitis infected dog

The nematode Dirofilaria immitis and its endosymbiont Wolbachia are the agents of canine heartworm (HW) disease. The worm´s main habitats are the pulmonary arteries, which elicit inflammation and disorders of the coagulation process. Clinical signs vary according to multiple factors, including worm burden, individual reaction, and duration of infection. This report describes an unusual case of pulmonary thromboembolism secondary to HW infection. A 7-year-old female dachshund presented with a history of cough, dyspnea, and two syncope episodes. On physical examination, the oral mucosa was slightly discolored, the femoral pulse was weak, respiratory distress was present, and the abdomen distended. Echocardiography showed severe right atrioventricular and pulmonary artery enlargement and a large structure with high echogenicity in the artery. After the dog died naturally, the lungs and heart were examined. Only one worm was found in the right ventricle, and a large thrombus was found in the main pulmonary artery. Histologically, fibrin thrombi and fragments of parasites were present inside the pulmonary artery branches. These findings highlight the importance of a thorough clinical evaluation of HW patients and confirm that only a few worms can threaten an animal’s life. Therefore, veterinarians must enforce chemoprophylaxis once treatment is life threatening.

P689 Surgical treatment of a right heart thrombus-in-transit with massive bilateral pulmonary embolism

Abstract Introduction Right heart thrombi are rare, found in up to 20% of pulmonary emboli (PE), and associated with significantly increased mortality(1). A thrombus entrapped in a PFO is a rare form of right heart thromboembolism. Clinical Case A 73-year-old male patient who had dyspnea for 10 days, was transferred to our hospital for the surgical treatment of a cardiac tumor. We performed TTE which revealed a free floating, huge mass( measuring more than 8 cm long) in the right atrium, that protruded in the right ventricle, with high risk of embolization. Another smaller mass, attached to the interatrial septum. Severe right ventricular dysfunction and severe pulmonary hypertension were present. Contrast-enhanced computer tomography was performed, which revealed severe bilateral pulmonary artery emboli with complete occlusion of right pulmonary artery branch. Clinical and paraclinical data strongly suggested that the huge cardiac mass was a thrombus that originated from the lower extremity veins. TEE showed that the thrombus was entrapped through the PFO, with a smaller part in the left atrium and the biggest portion in the right atrium. The patient underwent an emergent on-pump surgical cardiac and right pulmonary artery embolectomy. The right atrium was opened and a huge intracardiac thrombus with a lengh of 14 cm extending from the coronary sinus, to PFO in the left atrium and also in the right ventricle was removed. The right branch of the pulmonary artery was opened and a large volume of clot- 9 cm long- was removed. The patient was removed from cardio-pulmonary by-pass (CPB) on high doses of norepinephrine and dobutamine and necessitated initiation of venous-arterial ECMO to support the severe right heart dysfunction. He was extubated after 10 days, with little improvement in the clinical status. Transthoracic echocardiography showed smaller right heart cavities), normal left ventricular function, but persistent severe RV dysfunction and severe pulmonary hypertension. In the thirteenth postoperative day, he installed cardio-respiratory arrest and he died. Discussion In this report we describe a case of a patient with a huge intracardiac thrombus, entrapped through a PFO, associated with massive pulmonary embolism, with late presentation in our hospital and severe refractory right heart dysfunction. He underwent successful embolectomy, which is a unique procedure in the treatment of an acute pulmonary embolism and entrapped thrombus in a PFO. Conclusion The treatment of choice for emboli-in-transit is controversial. In a recent review, surgical thromboembolectomy showed a trend toward improved survival and significantly reduced systemic emboli compared to anticoagulation. Thrombolysis in these patients may cause fragmentation of thrombus and systemic embolization, resulting in increased mortality. Management decisions should be made with multidisciplinary coordination and consideration of complicating factors such as PFO. Abstract P689 Figure. Extensive biatrial thrombus

P1721 Reversible heart right failure. Pulmonary hypertension induced by Tyrosine Kinase Inhibitors

Abstract BACKGROUND Pharmacologically induced pulmonary hypertension (PH) is infrequent nowadays and it is included in the type 1 of the classification of PH. Tyrosine kinase inhibitors (TKI) are the cornerstone of the treatment of many haemotopoietic stem cell diseases. Dasatinib is a second-generation TKI used in chronic myeloid leukemia (CML) and as an infrequent cardiovascular side-effect (< 0,50%) could induce PH, usually reversible but life threatening. Only a few case series are published. CASE DESCRIPTION: We present a 51-year-old woman who was diagnosed of a CML when she was 46. Initially, she underwent therapy with imatinib but after 5 years of treatment she developed resistance to this drug, and dasatinib was prescribed as a second line drug. After 3 months of continuous treatment, she started with dry cough and effort dyspnea. Blood analysis, EKG and Chest X-Ray were made but did not show outstanding findings. An unspecific viral infection was the final diagnosis. The patient clinical condition deteriorated with major dyspnea and edemas in the lower limbs. A TTE showed moderate tricuspid regurgitation and severe HP systolic pulmonary artery pressure (sPAP) of 80 mmHg. The pulmonary acceleration time was shortened and a mesosistolic knock was present. Systolic dysfunction of the right ventricle and pericardial effusion (image 1,2,3,4) were noted. The right atrium was not dilated. Cava vein was dilated but with inspiratory collapse >50%. The left ventricular function was preserved, but first degree diastolic dysfunction was found. Other causes of PH were excluded (types 2, 3, 4). A CT pulmonary angiogram did not show segmental perfusion defects. Finally, a right heart catheterization confirmed the TTE findings: severe precapillary PH without postcapillary component. After the diagnosis was confirmed, TKI was stopped and double targeted therapy with ambrisentan + tadalafil was started. After 6 months of treatment a new TTE was made with complete reversal of the secondary changes in the myocardium induced by the PH. No tricuspid regurgitation was detected nor any indirect sign of PH was found. (image 5,6). CONCLUSIONS Drug-induced PH is rare nowadays and most cases were described in the seventies in the USA related with the epidemic of anorexigenic drugs. Although the pathogenesis still remains unclear, treatment includes immediately stopping the offending agent. Echocardiography due to its accessibility, reproducibility, consistence and low cost should be the first diagnostic tool to be considered, because as it is known, in the early stages of the disease, before developing right disfunction, clinical and conventional tests are non-specific. Abstract P1721 Figure. Echo images: previous and afte treatment

Organ changes found by postmortem examination in COVID-19 patients

The postmortem study results presented in 20 sources of literature on 186 COVID-19 patients were analyzed. Pathological changes were noted in multiple organs, by involving predominantly the respiratory, circulatory, and excretory systems. The changes in the lungs were characterized by an increase in organ weight (59.3%), a dark red color of the parenchyma (47.4%), compaction of lung tissue (56%), and signs of lung congestion (37.3%). The histological characteristics found during postmortem lung tissue examination, which were indicative of diffuse alveolar damage, were proliferation of type II alveolocytes in 65.2% of cases, the appearance of hyaline membranes lining the alveoli in 64.4%, and interstitial edema in 54.2%. In the analyzed sources, 22% of cases were noticed to have severe thrombosis and pulmonary artery branch embolism that was associated with lower extremity deep vein thrombosis. In all the sources of literature, acute tubular necrosis, tubular luminal dilatation, and interstitial edema were detected in the kidneys.

Subarachnoid block in transurethral surgery for bladder tumor in Eisenmenger’s syndrome

Uncorrected left to right intracardiac shunts can progress to a reversal of shunting in the fourth or fifth decade of life due to severe pulmonary artery hypertension. As the changes in the pulmonary vasculature are irreversible at this stage the condition can only be palliated by medication. Regional anaesthesia decreases the systemic vascular resistance and increases the right to left shunt but in optimal doses can minimize this shunting while protecting from other adverse effects of general anaesthesia. We highlight the anaesthetic management of a 53 year old male with Eisenmenger’s syndrome with atrial septal defect on anti failure medications successfully managed with a low dose subarachnoid blockade for transurethral surgery. We believe that this simplified management while providing a safe protocol for use.

Congenital right ventricular diverticulum: An atypical presentation.

Congenital ventricular diverticulum is a rare congenital malformation of the heart. It remains mostly asymptomatic unless associated with other congenital cardiac defects. We present a case of a 4-month-old child who had dextrocardia, large inlet ventricular septal defect, amounting to single ventricle, and severe pulmonary artery hypertension. There was a right ventricular diverticulum which passed through the foramina of Morgagni to give rise to a pulsating lump in the epigastrium. Right ventricular diverticulum presenting with a pulsating mass in the abdomen is a rare entity. The confirmation of diverticulum was made with histopathology.

Middle aortic syndrome coexisting with rheumatic mitral valve disease

Middle aortic syndrome (MAS) is a rare clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. Although rarely diagnosed and reported, it is a cause of hypertension in the middle aged. Its coexistence with rheumatic mitral valve disease is yet to be documented in the English literature. A 25-year-old female who had previously been managed as a hypertensive presented with difficulty with breathing, NYHA class II, and generalized body weakness, more in the lower limbs. General examination was normal. Her vitals were as follows: temperature- 98.5°F, pulse rate - 100/min, blood pressure (BP) - 160/100 mmHg (left upper limb), respiratory rate - 20/min, saturation - 100% on room air. Echocardiography revealed a thickened aortic valve, concentric left ventricular hypertrophy, thickened and prolapsed anterior mitral leaflet, thickened and fixed posterior mitral leaflet, severe mitral regurgitation, dilated left chambers, severe tricuspid regurgitation, and severe pulmonary artery hypertension (PAH, RVSP = 82 mmHg). She was scheduled for mitral valve replacement (MVR) and tricuspid valve repair. The diagnosis of surgical hypertension was missed. As a routine protocol in our institution, radial and femoral cannulation was done for invasive BP monitoring. Following such cannulation, radio-femoral pressure gradient was noted, with a suspicion of aortic coarctation, necessitating the need to defer the surgery. On further evaluation, computed tomography (CT) chest and CT angiography were suggestive of MAS extending from T8 to T11 thoracic vertebrae, with narrow-lumen proximal abdominal aorta. She subsequently had aorto-aortic bypass + MVR + tricuspid valve sutural annuloplasty via median sternotomy + laparotomy for definitive treatment. MAS is a rare vascular disorder. Of more rarity is its coexistence with rheumatic valvular disease complicated by severe PAH. With standard institutional protocols of evaluation, and a high index of suspicion, a missed diagnosis can be forestalled.

TRICUSPID VALVE (TV) LEAFLET EXPANSION IS THE MAIN ADAPTIVE CHANGE TO MAINTAIN COMPETENCY: A THREE-DIMENSIONAL ECHOCARDIOGRAPHY (3DE) STUDY IN A NOVEL CHRONIC RIGHT VENTRICULAR (RV) PRESSURE AND VOLUME LOADED PIGLET MODEL

Tricuspid valve regurgitation (TR) develops in 25-35% of children with hypoplastic left heart syndrome (HLHS), a risk factor for morbidity and mortality. Surgical strategies for TR in HLHS are modest in success and durability, therefore warrants improved understanding and innovation. We developed a novel piglet model simulating infant HLHS RV physiology to study TV adaptation to chronic increased preload and afterload. We hypothesize that TV competency is maintained by adaptive rapid leaflet expansion despite annular dilation. Sixteen piglets at 4-5weeks of age (maturity equivalent to infants) underwent left thoracotomy. Intervention piglets (IP, n=8) had their pulmonary valve torn to produce moderate to severe pulmonary regurgitation (volume loading) and pulmonary artery band placed to increase RV pressure. Control piglets (CP, n=8), age and gender matched, had sham surgery. Following a 4-week recovery, all piglets underwent hemodynamic assessment and 3DE of TV. We assessed TV annulus and leaflet geometry in mid-systole using a custom 3DE software (MATLAB). Ex-vivo TV total leaflet area was measured. Comparisons between IP and CP were made using Student t-test with significance at p < 0.05. Values were expressed as mean (standard deviation). IP had significant pulmonary regurgitation (mean PR reverse/forward velocity time integral ratio 0.68 (0.09)) and mean RV systolic pressure 77.9% of systemic pressure, reflected in thicker RV free wall and anterior papillary muscle, consistent with an effective model. IP and CP were similar in weight and TR grade. IP TV annulus were larger (p=0.02), more circular (p=0.03) and leaflets more tethered (p=0.02) with 43% larger total leaflet area (p=0.01) [see Table]. Greatest expansion was seen in the posterior leaflet (p=0.01). 3DE TV total leaflet area correlated with ex-vivo TV pathologic specimen area (r=0.63, p=0.02). Following chronic exposure to increased RV preload and afterload, there is TV annular dilation and likely unchanged sub-valve apparatus, resulting in leaflet tethering. In-vivo 3DE identified rapid leaflet expansion as the main adaptive change to maintain competency from detrimental remodeling. Further research into modulation of TV leaflet growth may be important for understanding TV failure in HLHS.

My Heart Is Failing and I Am Pregnant

A 34-year-old, gravida 2, para 0, woman who was at 29 4/7-weeks gestation. The patient had a history of rheumatic heart disease and mitral stenosis with severe pulmonary artery hypertension (New York Heart Association Heart Failure Class III). A 34-year-old woman, gravida 2, para 0 who was at 29 4/7-weeks gestation. The patient had a history of rheumatic heart disease and mitral stenosis with severe pulmonary artery hypertension (New York Heart Association Heart Failure Class III); she presented for admission for medical management with worsening symptoms of heart failure. Care management of this complex patient required multiple medical and nursing disciplines to ensure an optimal outcome. From a nursing perspective, during the antepartum course, the patient was managed by our antepartum nurses; however, she required cardiac telemetry, which was monitored remotely by a telemetry-certified nurse. As the physicians collaborated and their plan unfolded, gaps were identified by nursing leadership. These gaps included information sharing regarding patient acuity, potential for mortality, resources required for birth and the immediate postpartum period, and lack of a compressive emergency plan for deteriorating cardiac status. Cardiac nursing and obstetric nursing leadership were instrumental in compelling all medical disciplines to come together in a formal setting to discuss the patient’s plan of care for birth and the immediate postpartum period. It is critical to identify comorbidities during patient assessment and the determination of nursing interventions, recognizing that some interventions go beyond the scope of cardiac or obstetric nurses and must be done collaboratively.

Laron syndrome related to homozygous growth hormone receptor c.784&gt;C mutation in a patient with hypoplastic pulmonary arteries

Laron syndrome, also known as growth hormone insensitivity, is an autosomal recessive disorder characterised by short stature due to mutations or deletions in the growth hormone receptor (GHR), leading to congenital insulin-like growth factor 1 (IGF1) deficiency. Cardiac abnormalities, such as patent ductus arteriosus or peripheral vascular disease are rare in patients with Laron syndrome, but cardiac hypertrophy has been observed after IGF1 therapy. In this report, we present a 10-year-and-5-month-old girl with severe peripheral-type pulmonary artery hypoplasia and Laron syndrome related to homozygous GHR c.784>C mutation.

The role of extracorporeal life support in the management with severe idiopathic pulmonary artery hypertension undergoing lung transplantation: are those patients referred too late?

Idiopathic pulmonary artery hypertension (iPAH) is a relatively minor indication for lung transplantation (LTx) with comparatively poorer outcomes. Extracorporeal life support (ECLS) in various forms is increasingly being used in the management of this entity. However, the data and experience with this therapy remains limited. We evaluated the role of ECLS in the management of severe iPAH patients as a bridge to LTx as well as post LTx support. A retrospective analysis of iPAH patients that received LTx between January 2007 and May 2014 was performed. Early- and mid-term outcomes were analyzed for this patient cohort. Also, early and mid-term outcomes after LTx were compared to the control group of patients with other diagnoses using unadjusted analysis and 1:3 propensity score matching. Of 321 LTx performed during the study period in our centre 15 patients had iPAH as a cause of end-stage lung disease. Four iPAH (27%) patients were bridged to LTx utilizing ECLS in the form of veno-arterial ECMO and extra-corporeal CO2 removal device, whereas 9 patients (60%) required ECLS support for primary graft dysfunction (PGD) after surgery. Patients with iPAH required more frequently on-pump LTx, both pre and post LTx ECLS, and had significantly lower pO2/FiO2 ratio at 24, 48 and 72 hours after LTx. Also iPAH patients had significantly longer ICU and hospital stay. Whereas the incidence of postoperative bronchiolitis obliterans syndrome (BOS) and rejection was comparable to the control group, overall cumulative survival with up to 6 years follow-up was significantly poorer in the iPAH group. After propensity score matching, the results in terms of postoperative outcomes remained as in the unadjusted analysis. ECLS is an essential tool in the armamentarium of any lung transplant program treating iPAH with a potential of bridge patients to transplantation and to overcome graft dysfunction after LTx. Despite utilization of ECLS in the management of iPAH, the outcomes in terms of primary graft failure and survival remain poor compared to patients with other diagnoses.

Prophylactic Right Ventricular Assist Device for High-Risk Patients Undergoing Valve Corrective Surgery

BackgroundRight ventricular failure (RVF) after cardiac surgery is associated with poor outcomes. Treatment commonly consists of afterload reduction, contractility optimization, and systemic vasopressors. The aim of this study was to propose a novel strategy of prophylactic right ventricular assist device (RVAD) insertion during valve corrective surgery for patients at high risk for RVF. MethodsBetween 2014 and 2017, 10 consecutive patients at high risk for RVF (severe baseline right ventricular dysfunction or systemic pulmonary artery pressures) underwent valve reconstructive surgery with prophylactic RVAD insertion. We reviewed patient characteristics and outcomes. ResultsAll 10 patients had successful RVAD insertion, support and wean, and survival to hospital discharge. Generally, the right ventricle showed echocardiographic evidence of worsening function perioperatively but recovery of function at the time of follow-up. Patients required minimal inotropic support, and no patients required extracorporeal membrane oxygenation. Major complications included prolonged mechanical ventilation (n = 4), metabolic encephalopathy (n = 1), and sternal wound infection (n = 2). At a mean follow-up of 445.1 ± 230.9 days, 7 of 8 patients had clinically New York Heart Association functional class 1 (n = 7), and 1 patient had New York Heart Association functional class 2 (n = 1). There were 2 late mortalities. ConclusionProphylactic RVAD insertion may be useful in supporting patients at high risk for RVF perioperatively when undergoing high-risk valve corrective surgery. Further investigation is warranted.

Chronic myocardial damage in idiopathic pulmonary arterial hypertension: a clinical case

Chronic myocardial injury, determined by a constantly elevated cardiac troponin (cTn) value, may occur in patients with pulmonary arterial hypertension (PAH), indicating the severity of the disease and associated with an adverse prognosis. Increasing of cTn among patients with PAH was registered prior to 2018, but after the Fourth universal definition of myocardial infarction (2018) was released the understanding of this phenomenon has been changed. One of the causes of chronic myocardial injury in patients with PAH is the compression of coronary arteries. Left main coronary artery (LMCA) compression is an uncommon and probably underestimated cause for angina-like chest pain and ventricular dysfunction in patients with severe pulmonary artery hypertension. LMCA compression by an enlarged pulmonary artery trunk has been associated with angina, but appropriate diagnostic and treatment approaches remain poorly defined. This is a case report on a woman with idiopathic PAH and compression of the LMCA by an enlarged pulmonary artery. The diagnosis of LMCA stenosis was confirmed by multi-slice computed tomography and coronary angiography. Biomarkers such as cTn and brain natriuretic peptide have also been identified as criteria of optimal treatment. Percutaneous coronary intervention in conjunction with PAH-specific therapy appears to be a feasible, safe, and effective treatment option for patients with extrinsic compression of the LMCA from pulmonary artery enlargement

Neuregulin-1 attenuates right ventricular diastolic stiffness in experimental pulmonary hypertension.

We have previously shown that treatment with recombinant human neuregulin-1 (rhNRG-1) improves pulmonary arterial hypertension (PAH) in a monocrotaline (MCT)-induced animal model, by decreasing pulmonary arterial remodelling and endothelial dysfunction, as well as by restoring right ventricular (RV) function. Additionally, rhNRG-1 treatment showed direct myocardial anti-remodelling effects in a model of pressure loading of the RV without PAH. This work aimed to study the intrinsic cardiac effects of rhNRG-1 on experimental PAH and RV pressure overload, and more specifically on diastolic stiffness, at both the ventricular and cardiomyocyte level. We studied the effects of chronic rhNRG-1 treatment on ventricular passive stiffness in RV and LV samples from MCT-induced PAH animals and in the RV from animals with compensated and decompensated RV hypertrophy, through a mild and severe pulmonary artery banding (PAB). We also measured passive tension in isolated cardiomyocytes and quantified the expression of myocardial remodelling-associated genes and calcium handling proteins. Chronic rhNRG-1 treatment decreased passive tension development in RV and LV isolated from animals with MCT-induced PAH. This decrease was associated with increased phospholamban phosphorylation, and with attenuation of the expression of cardiac maladaptive remodelling markers. Finally, we showed that rhNRG-1 therapy decreased RV remodelling and cardiomyocyte passive tension development in PAB-induced RV hypertrophy animals, without compromising cardiac function, pointing to cardiac-specific effects in both hypertrophy stages. In conclusion, we demonstrated that rhNRG-1 treatment decreased RV intrinsic diastolic stiffness, through the improvement of calcium handling and cardiac remodelling signalling.

AN UNUSUAL CAUSE OF PULMONARY HYPERTENSION

SESSION TITLE: Pulmonary SESSION TYPE: Global Case Reports PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM INTRODUCTION: Unilateral absence of left pulmonary artery (UAPA)is a rare congenital abnormality with variable presentation. Patients with isolated UAPA can remain symptomatic in to late adulthood.Unilateral absence of pulmonary artery is a rare congenital malformation that may develop in isolation or in association with congenital cardiovascular anomalies. 1,2Associated cardiovascular anomalies may include Tetralogy of Fallot (TOF), atrial septal defect, coarctation of the aorta, a right-sided aortic arch, truncus arteriosus, and pulmonary atresia .In reported cases of isolated UAPA incidence has been reported to range from 1 in 200,000 to 1 in 300,000. 3 Agenesis of the right pulmonary artery has a greater incidence than agenesis of the left .UAPA is believed to result from the involution of the proximal sixth aortic arch, thereby forgoing expected embryological fusion with the pulmonary trunk . Most common symptoms experienced include recurrent pulmonary infections, hemoptysis, chestpain and shortness of breath upon exertion .We present a case of 45 yrs old female referred for evaluation of unexplained exertion breathlessness found to have unilateral absent left pulmonary artery. CASE PRESENTATION: Middle age lady with moderate persistent asthma on optimised inhaler medications, referred for evaluation of unexplained exertion breathlessness.Patient had longstanding history of exercise intolerance relived by rest. X ray chest showed enlarged right pulmonary artery with prominent vascular markings. A Multi detector Computed Tomography (MDCT) pulmonary angiogram showed dilated main pulmonary artery with absent left pulmonary artery. Echocardiogram revealed severe Tricuspid regurgitation with severe pulmonary artery hypertension. Her pulmonary function test showed modern obstructive pattern. she was started on diuretics and vasodilators along with inhaler medications. DISCUSSION: Left-sided agenesis is a rare entity that occurs half as frequently as right-sided agenesis. Approximately 80% of left-sided agenesis is associated with a congenital cardiovascular malformation necessitating medical attention in early life .The other subset of isolated left pulmonary artery agenesis without associated cardiovascular anomaly, presenting later on in life.The treatment for UAPA is patient specific and will vary depending on age of presentation, associated cardiovascular anomalies, and symptomatology. The options in young patients include revascularization of the distal affected pulmonary artery.Lobectomy, or selective embolization of systemic arterial supply, may be considered for patients with hemoptysis . Asymptomatic adult patients with severe pulmonary hypertension may be started on vasodilators.Regular follow-up and observation of pulmonary hemodynamics is recommended CONCLUSIONS: Early diagnosis and treatment of pulmonary hypertension is required is a serious complication. Reference #1: 1. Aypak C, Yikilkan H, Uysal Z, Gorpelioglu S: Unilateral absence of the pulmonary arteryincidentally found in adulthood. Case Rep Med. 2012, 2012:1-3. Accessed: 2/1/2016:http://www.hindawi.com/journals/crim/2012/942074/. 10.1155/2012/942074. Reference #2: Ghanbari H, Feldman D, David S, Saba S: Unilateral absence of a left pulmonary artery:successful therapeutic response to a combination of bosentan and warfarin. CircaCardiovascImaging. 2009, 2:e46-e48. 10.1161/CIRCIMAGING.108.825745 Reference #3: Kruzliak P, Syamasundar RP, Novak M, Pechanova O, Kovacova G: Unilateral absence ofpulmonary artery: pathophysiology, symptoms, diagnosis and current treatment. ArchCardiovasc Dis. 2013, 106:448-454. 10.1016/j.acvd.2013.05.004. DISCLOSURES: No relevant relationships by Nagarajan Appusamy, source=Web Response

A rare cause for aphonia - Ortners syndrome

&lt;p&gt;Rheumatic mitral stenosis is prevalent in the developing world and presents with a wide array of manifestations. Aphonia and hoarseness of voice secondary to recurrent laryngeal nerve paralysis (Ortner’s syndrome) is an uncommon manifestation of mitral stenosis. We present a case severe mitral stenosis with moderate to severe tricuspid valve regurgitation and severe pulmonary artery hypertension that was masquerading as aphonia and hoarseness of voice for eight months. Mitral valve replacement and tricuspid annuloplasty was done and the quality of voice improved post-surgery. &lt;/p&gt;

Are Downhill Varices an Overlooked Entity of Upper Gastrointestinal Bleedings?

Aim Downhill varices are not so safe as thought and can lead to life-threating or mortal bleeding complication, even if rare. In order to draw attention to this topic, we analysed 129 patients. Materials and Methods We evaluated the electronic endoscopy data records of all patients undergoing upper gastrointestinal endoscopy over a nine-year period from January 2004 till December 2012, within a retrospective approach. The primary endpoints, incidence, causes, kind of resulting upper gastrointestinal bleeding, and the severity of the bleeding were evaluated. Secondary endpoints were the evaluation of the size of downhill varices and a comparison of the risk of bleeding between downhill varices and uphill varices. Results Downhill varices were identified, described, and/or documented in 129 patients of 25,680 upper gastrointestinal endoscopies. 26 patients had central venous catheter or port implantation, 22 patients had a history of an implantation of a cardiac pacemaker, 7 patients had severe pulmonary artery embolism, and 4 patients had severe chronic obstructive pulmonary disease. Two patients had mediastinal tumors, and one patient had a large retrosternal goiter as a possible cause of the vena cava syndrome. Altogether, 62 patients were related to a vena cava superior syndrome; 67 were not. Conclusions Downhill varices can be seen with an incidence of 0.5%. Therapeutic means are the banding therapy as a safe and effective option. Severe bleedings associated with downhill varices can be mortal. Severe forms of downhill varices should be examined in relation to the origin in order to start a specific therapy.

Mitral Valve Replacement in Severe Pulmonary Artery Hypertension — a Single Center Single Surgeon Experience

Mitral Valve Replacement in Severe Pulmonary Artery Hypertension — a Single Center Single Surgeon Experience

Predictive Model for Secundum Atrial Septal Defect Closure with Pulmonary Artery Hypertension in Adult: When to Close.

Secundum atrial septal defect (SASD) closure is contraindicated in the presence of severe pulmonary artery hypertension (PAH). However, there is no consensus on the threshold of severe PAH, in terms of mean pulmonary artery pressure (mPAP), which would contraindicate for defect closure surgery in adults. Furthermore, PAH can persist, or even increase in severity, after the closure. The aim of this study was to produce a predictive model correlating mPAP in adult SASD patients with PAH after defect closure surgery.Methods: Between January 2014 and March 2017, 29 consecutive adult SASD patients who had PAH and underwent defect closure surgery were included in the study. Age, right atrium (RA) dimension, right ventricle (RV) dimension, and mPAP before surgery were analyzed using multiple regression to produce the model. Multiple regression produced the following model: mPAP prediction = (0.24)(Age) + (0.06)(mPAP before surgery) + (0.17)(RA dimension) + (0.47)(RV dimension) - 13.79 (P = .0008). The mPAP prediction was compared to mPAP of the patients six to nine months after surgery, and showed no significant difference (P = .9562).Conclusion: In adult SASD patients with PAH, our model can significantly predict the mPAP after the closure. If the predicted mPAP is within its normal range, the closure is indicated.

Incidence, Timing, Causes and Predictors of Early and Late Re-Hospitalization in Patients Who Underwent Percutaneous Mitral Valve Repair With the MitraClip System

The pattern and reasons for re-hospitalization (RH) after MitraClip implantation are not well characterized. A total of 322 consecutive MitraClip patients were included, with data stratified by RH status. Multivariate analyses were conducted to identify predictors of early (30-day) and late (30-day to 12-month) RH. Eighty-nine patients (27.6%) were readmitted to hospital during the study period and early RH occurred in 27%. The median time from MitraClip to RH was 99 days. RH was mostly related to cardiovascular causes (66.3%). Anemia and gastrointestinal bleeding were the most frequent noncardiovascular causes. Independent predictors of early RH were length of stay ≥3 days during the index procedure (odds ratio [OR] 4.13, 95% confidence interval [CI] 1.32 to 12.91), reduction of left ventricular ejection fraction ≥5% after MitraClip implantation (OR 4.88, 95% CI 1.36 to 18.91), and severe systolic pulmonary artery pressure ≥60 mmHg at discharge (OR 3.72, 95% CI 1.23 to 11.26). Conversely, the independent predictors of late RH were device failure (OR 4.02, 95% CI 1.22 to 13.25) and systolic pulmonary artery pressure ≥60 mmHg at discharge (OR 2.34, 95% CI 1.01 to 5.44). In patients with early RHs, survival was significantly worse at 12 months compared with patients with late RH and no-RH (69.3% vs 82.6% vs 86%, p <0.001). In conclusion, RH is not uncommon after MitraClip implantation and cardiovascular causes represent its most frequent etiology. Clinical and echocardiographic predictors of early and late RH can be identified at discharge. Early RH carries a worse prognosis than late RH.