A novel percutaneously adjustable device for ligature of the vertical vein in the setting of obstructive totally anomalous pulmonary venous connection

2007 ◽  
Vol 17 (4) ◽  
pp. 380-386 ◽  
Author(s):  
Ujjwal K. Chowdhury ◽  
Anand Mishra ◽  
Anita Saxena ◽  
Shyam S. Kothari ◽  
Amber Malhotra ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Heart Failure ◽  
New Device ◽  
Computed Tomographic ◽  
Vertical Vein ◽  
Pulmonary Arterial ◽  
Hypertensive Crises ◽  
Anomalous Pulmonary Venous Connection

AbstractBackgroundA patent vertical vein might be desirable in patients with obstructive totally anomalous pulmonary venous connection with pulmonary hypertension, in order to decrease perioperative pulmonary arterial pressure and avoid pulmonary hypertensive crises. A subset of patients with an unligated vertical vein requires interruption of the vein due to the development of significant left-to-right shunt and right heart failure. We describe here a new device, permitting adjustable ligation of the vertical vein, which permits us to avoid multiple reoperations.Patients and methodsIn five patients, aged 2, 4, 3, 4, and 3 months respectively, and undergoing rechannelling of totally anomalous pulmonary venous connection with an unligated vertical vein, were treated with a device permitting adjusted ligation of the vertical vein over the course of postoperative congestive cardiac failure.ResultsThere was no early or late death. Postoperatively, all ligatures were tightened gradually over a period of 24 to 96 hours, maintaining stable haemodynamics. At a mean follow-up of 55.40 months, there was no evidence of congestive heart failure in any patient, the clinical risk score varying from zero to 2, and no requirement of anti-failure medications. Computed tomographic angiograms during follow-up revealed absence of flow through the vertical vein, and ruled out distortion of the left upper pulmonary and left brachiocephalic veins.ConclusionUse of a percutaneously adjustable device to ligate the vertical vein allows gradual tightening or loosening of the ligature under optimal physiologic conditions, without re-opening the sternum, or having to resort to another thoracotomy once the reactive components of pulmonary hypertension disappear.

scholarly journals Critical Care Management of Decompensated Right Heart Failure in Pulmonary Arterial Hypertension Patients – An Ongoing Approach

2021 ◽  
Vol 7 (3) ◽  
pp. 170-183
Author(s):  
Ioan Tilea ◽  
Andreea Varga ◽  
Anca-Meda Georgescu ◽  
Bianca-Liana Grigorescu
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Care Management ◽  
Right Heart Failure ◽  
Right Heart ◽  
Continuous Increase ◽  
Pulmonary Arterial

Abstract Despite substantial advancements in diagnosis and specific medical therapy in pulmonary arterial hypertension patients’ management, this condition continues to represent a major cause of mortality worldwide. In pulmonary arterial hypertension, the continuous increase of pulmonary vascular resistance and rapid development of right heart failure determine a poor prognosis. Against targeted therapy, patients inexorable deteriorate over time. Pulmonary arterial hypertension patients with acute right heart failure who need intensive care unit admission present a complexity of the disease pathophysiology. Intensive care management challenges are multifaceted. Awareness of algorithms of right-sided heart failure monitoring in intensive care units, targeted pulmonary hypertension therapies, and recognition of precipitating factors, hemodynamic instability and progressive multisystem organ failure requires a multidisciplinary pulmonary hypertension team. This paper summarizes the management strategies of acute right-sided heart failure in pulmonary arterial hypertension adult cases based on recently available data.

Life-threatening complications of pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2497-2500
Author(s):  
Marion Delcroix ◽  
Catharina Belge
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Coronary Artery ◽  
Pulmonary Arterial Hypertension ◽  
Severe Disease ◽  
Right Heart Failure ◽  
Artery Dissection ◽  
Life Threatening ◽  
Pulmonary Arterial ◽  
Arterial Dilation

Pulmonary arterial hypertension is a severe disease with a 1-year mortality of over 10%. Since it is a rare disease, it is usually managed in expert centres. However, life-threatening complications can occur at any moment and place and should be adequately taken care of to prevent avoidable deaths. Therefore, based on an analysis of the causes of death, this chapter describes the most important complications and their management: right heart failure, arrhythmias, haemoptysis, pericardial effusion, pulmonary arterial dilation with as a consequence left main coronary artery compression, and pulmonary artery dissection, as well as treatment-related adverse events.

scholarly journals Jobb kamrai adaptáció pulmonalis artériás hypertoniában

2021 ◽  
Vol 162 (37) ◽  
pp. 1485-1493
Author(s):  
Györgyi Csósza ◽  
Zsófia Lázár ◽  
Zsolt Rozgonyi ◽  
Hajnalka Vágó ◽  
György Losonczy ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Ventricular Dysfunction ◽  
Right Heart Failure ◽  
Progressive Increase ◽  
Right Heart ◽  
Cardiac Adaptation ◽  
The Face ◽  
Pulmonary Arterial ◽  
The Right

Összefoglaló. Pulmonalis artériás hypertoniában (PAH) a tüdőartériák falának átépülése az elsődleges patofiziológiai eltérés, amely a pulmonalis vascularis rezisztencia (PVR) és a pulmonalis nyomás progresszív emelkedéséhez vezet. Ez a nyomásemelkedés a jobb szívfélben az afterload fokozódásához vezet, ami hosszú távon jobbkamra-diszfunkciót és jobbszívfél-elégtelenséget okoz. Az egyre növekvő PVR mellett kialakuló cardialis adaptáció pontos patomechanizmusa nem ismert, de egyes betegek esetén nagyon eltérő lehet az adaptáció mértéke és kialakulásának üteme. A kialakuló myocardium-hypertrophia és -dilatáció mértéke nagyban függ a PAH etiológiájától, de emellett egyéb tényezők – mint az életkor, a neurohumoralis aktiváció mértéke, genetikai és epigenetikai faktorok – is jelentősen befolyásolják. Minél kevésbé képes a jobb kamra megtartani funkcióját az egyre növekvő ellenállással szemben, annál gyorsabban alakul ki a jobbszívfél-elégtelenség, és annál rosszabbak a beteg életkilátásai. Ezen folyamatok jobb megismerése klinikai jelentőséggel bír, mivel a jobb kamrai adaptáció elősegítése javíthatja a betegség kimenetelét. Orv Hetil. 2021; 162(37): 1485–1493. Summary. Remodeling of the pulmonary artery wall is the primary pathophysiological abnormality in pulmonary arterial hypertension leading to a progressive increase in pulmonary vascular resistance (PVR) and pulmonary arterial pressure. The elevation of pressure increases the afterload in the right heart, causing right ventricular dysfunction and right heart failure in the long term. The exact pathomechanism of cardiac adaptation with increasing PVR is unknown, but the degree and rate of adaptation may be very different in patients suffering from pulmonary hypertension. The development of myocardial hypertrophy and dilatation is highly dependent on the etiology of pulmonary hypertension, but is also significantly influenced by other factors such as age, degree of neurohumoral activation, and genetic and epigenetic factors. Right heart failure develops and life expectancy shortens if the right ventricle is unable to maintain its function in the face of increasing resistance. Orv Hetil. 2021; 162(37): 1485–1493.

scholarly journals Lobectomy with bronchoplasty and pulmonary arterial angioplasty for lung cancer after correction of contralateral partial anomalous pulmonary venous connection

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Koichi Fukumoto ◽  
Madoka Goto ◽  
Yasuhisa Ichikawa ◽  
Yuta Kawasumi ◽  
Mika Uchiyama ◽  
...  
Keyword(s):  
Heart Failure ◽  
Lung Cancer ◽  
Pulmonary Resection ◽  
Lung Tumor ◽  
Primary Lung Cancer ◽  
Right Heart Failure ◽  
Contralateral Side ◽  
Pulmonary Arterial ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Abstract Background There have been few reports on surgically treated primary lung cancer accompanied by contralateral partial anomalous pulmonary venous connection (PAPVC). In such cases, repair of the PAPVC might be necessary to avoid postoperative right-heart failure due to the increased flow of the left-to-right shunt. Case presentation We herein report a case of lung adenocarcinoma treated by left-upper lobectomy with bronchoplasty and pulmonary arterial angioplasty after induction chemoradiation therapy followed by surgical correction of the PAPVC in the right-upper lobe. The patient is alive without recurrence of lung cancer or any symptoms of heart failure 17 months after pulmonary resection. Conclusion When considering performing major pulmonary resection for lung tumor, thoracic surgeons should pay close attention to the presence of a PAPVC not only on the ipsilateral side of the lung tumor, but also the contralateral side, although it is a rare phenomenon.

scholarly journals Potts Shunt in Children With Familial Primary Pulmonary Hypertension: A Case Report and Brief Literature Review

2021 ◽  
Vol 9 (3) ◽  
pp. 247-254
Author(s):  
Mahya Mobinikhaledi ◽  
◽  
Ali Arjmand Shabestari ◽  
Yazdan Ghandi ◽  
◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Medical Treatment ◽  
Right Heart Failure ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
The Right

Introduction: The primary concern about Familial Pulmonary Arterial Hypertension (FPAH) is the development of right heart failure, which ultimately leads to sudden death. Despite recent advances in pharmacological therapy, the mortality rate in children is still high, emphasizing the importance of novel treatments. Moreover, there is still no definitive treatment for children with severe pulmonary hypertension. The consequences of right heart failure led to the proposal of a surgical approach, the Potts shunt, to decompress the right ventricle, providing shunting of deoxygenated blood to lower extremities and improving overall cardiac output. This surgical technique creates an anastomosis between the left pulmonary artery and the descending aorta, providing a palliative treatment to off-load the right ventricle. Case Presentation: We report an 11-year-old girl with familial pulmonary arterial hypertension and right ventricular failure who benefited from a Potts shunt with good mid-term results. The patient was diagnosed at the age of 5 when she had a history of fainting and persistent syncope. The patient was under medical treatment therapy with bosentan, sildenafil, acetylsalicylic acid, and warfarin for six years. The results of molecular genetic testing, which was confirmed with direct sequencing of the Bone Morphogenetic Protein Receptor Type 2 (BMPR2) gene, revealed a heterozygous pathogenic mutation. Since she was diagnosed with Pulmonary Arterial Hypertension (PAH), she lost her grandmother, an aunt, and father because of PAH. Her 14-year-old sister also had mutated the BMPR2 gene without developing FPAH. Conclusions: The Potts shunt provides an interventional step for palliation of patients with familial pulmonary hypertension and severe right heart failure refractory to medical treatment. It opens the door to the possibility of lung transplantation in the future. We did not see any complications within 6 years after placing the Potts shunt.

scholarly journals Challenges in pulmonary hypertension: managing the unexpected

2015 ◽  
Vol 24 (138) ◽  
pp. 674-681 ◽  
Author(s):  
Karen M. Olsson ◽  
Massimiliano Palazzini
Keyword(s):  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Treatment Success ◽  
Right Heart Failure ◽  
High Dose ◽  
Triple Combination ◽  
Combined Approach ◽  
Right Heart ◽  
Pulmonary Arterial

The diverse challenges associated with diagnosis and management of patients with pulmonary hypertension are illustrated in this case-based review. Case 1 describes a patient diagnosed with pulmonary arterial hypertension (PAH) with right heart failure and active systemic lupus erythematosus who was effectively treated with an up-front triple combination of PAH therapies and immunosuppressive therapy. In case 2, a diagnosis of pulmonary veno-occlusive disease was reached after a combined approach of clinical suspicion, physical examination, and invasive and noninvasive tests. Cautious PAH therapy and high-dose diuretics provided clinical benefit in this patient and served as a bridge to lung transplantation. These cases highlight the need for ongoing follow-up of patients with PAH, comprising frequent assessment of treatment success and continued diagnostic evaluation.

Presence of Kidney Disease as an Outcome Predictor in Patients with Pulmonary Arterial Hypertension

2018 ◽  
Vol 47 (2) ◽  
pp. 134-143 ◽  
Author(s):  
Laurent Bitker ◽  
Florence Sens ◽  
Cécile Payet ◽  
Ségolène Turquier ◽  
Antoine Duclos ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Kidney Disease ◽  
Cardiac Index ◽  
Right Heart Failure ◽  
Female Gender ◽  
Systemic Hypertension ◽  
Right Heart ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) may lead to right heart failure and subsequently alter glomerular filtration rates (GFR). Chronic kidney disease (CKD, GFR <60 mL/min/1.73 m2) may also adversely affect PAH prognosis. This study aimed to assess how right heart hemodynamics was associated with reduced estimated GFR (eGFR) and the association of CKD with survival in PAH patients. Methods: In a prospective PAH cohort (2003–2012), invasive hemodynamics and eGFR were collected at diagnosis (179 patients) and during follow-up (159 patients). The prevalence of CKD was assessed at PAH diagnosis. Variables, including hemodynamics, associated with reduced eGFR at diagnosis and during follow-up were tested in multivariate analysis. The association of CKD with survival was evaluated using a multivariate Cox regression model. Results: At diagnosis, mean age was 60.4 ± 16.5 years, mean pulmonary arterial pressure was 43 ± 12 mm Hg, and eGFR was 74.4 ± 26.4 mL/min/1.73 m2. CKD was observed in 52 incident patients (29%). Independent determinants of reduced eGFR at diagnosis were age, systemic hypertension, and decreased cardiac index. Independent determinants of reduced eGFR during follow-up were age, female gender, PAH etiology, systemic hypertension, decreased cardiac index, and increased right atrial pressure. Age ≥60 years, female gender, NYHA 4, and CKD at diagnosis were independently associated with decreased survival. The adjusted hazards ratio for death associated with CKD was 1.81 (95% confidence interval [1.01–3.25]). Conclusion: CKD is frequent at PAH diagnosis and is independently associated with increased mortality. Right heart failure may induce renal hypoperfusion and congestion, and is associated with eGFR decrease.

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