Factors associated with systemic to pulmonary arterial collateral flow in single ventricle patients with superior cavopulmonary connections

Background:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiorgan involvement. Pulmonary hypertension (PH) is an uncommon manifestation with high morbidity and mortality whose characteristics, prevalence and evolution in SLE are not completely defined.Objectives:Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to to identify the factors associated with pulmonary hypertension (PH) in systemic lupus erythematosus (SLE).Methods:Prospective observational study on a multicenter Spanish inception cohort. Patients with SLE, diagnosed by the American College of Rheumatology (ACR) criteria, since January 2009, who had at least one transthoracic echocardiogram (TTE) performed were selected. Demographic data, diagnostic criteria, follow-ups, treatments and SLEDAI were analyzed.Results:Of 289 patients diagnosed with SLE with TTE performed, 15 (5.2%) patients were identified to have PH. Mean age was 56,9±7,7 years, of which 93,3% (14) were women and 80% (12) Caucasian. The ACR score at diagnosis was 4.66. Mean SLEDAI was 15. Only 5 patients had dyspnea at the time of diagnosis. Mean pulmonary arterial systolic pressure was 49.2±5.6 mmHg. Among the PH, 4 patients had pericarditis (26.6%), 3 (20%) valvulopathies (1 antiphospholipid syndrome), 1 patient pulmonary embolism and 1 shrinking lung. Multivariable analysis indicated that pericarditis (odds ratio (OR)=2.53), and valvulopathies (OR 8.96) were independently associated with the development of PH in SLE. Having PH was associated with older age at diagnosis (p<0.001), more dyspnea (p<0.001), higher ESR (p=0.007), more serositis (p<0.001), higher SLEDAI (p=0.011), higher SLICC (p <0.001), higher number of admissions (p=0.006) and higher mortality (p=0.003).Conclusion:PH in SLE is a serious comorbidity with high mortality. In the RELES cohort it was associated with increased disease activity, pericarditis and valvulopathies. Performing TTE in patients with SLE may favor early diagnosis and treatment.References:[1]Kim JS, Kim D, Joo YB, et al. Factors associated with development and mortality of pulmonary hypertension in systemic lupus erythematosus patients.Lupus. 2018;27(11):1769–1777.[2]Bazan IS, Mensah KA, Rudkovskaia AA, et al. Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.Respir Med. 2018;134:42–46.Disclosure of Interests:Jorge Álvarez Troncoso: None declared, Ángel Robles Marhuenda: None declared, Francesca Mitjavila Villero: None declared, Francisco José García Hernández: None declared, Adela Marín Ballvé: None declared, Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Gonzalo Salvador Cervelló: None declared, Eva Fonseca: None declared, Isabel Perales Fraile: None declared, Guillermo Ruiz-Irastorza: None declared

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Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening

European Respiratory Review ◽

10.1183/09059180.00006111 ◽

2011 ◽

Vol 20 (122) ◽

pp. 270-276 ◽

Cited By ~ 18

Author(s):

C. P. Denton ◽

E. Hachulla

Keyword(s):

Risk Factors ◽

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Factors Associated ◽

Pulmonary Arterial

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248 Factors associated with raised pulmonary arterial pressure on echocardiography

European Journal of Echocardiography ◽

10.1016/s1525-2167(05)80102-7 ◽

2005 ◽

Vol 6 ◽

pp. S27-S27

Keyword(s):

Arterial Pressure ◽

Pulmonary Arterial Pressure ◽

Factors Associated ◽

Pulmonary Arterial

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Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure

Pediatric Cardiology ◽

10.1007/s00246-020-02424-w ◽

2020 ◽

Vol 41 (8) ◽

pp. 1651-1659

Author(s):

Ida Jeremiasen ◽

Karin Tran-Lundmark ◽

Nikmah Idris ◽

Phan-Kiet Tran ◽

Shahin Moledina

Keyword(s):

Arterial Pressure ◽

Single Ventricle ◽

Pulmonary Arterial Pressure ◽

Vasodilator Therapy ◽

Single Ventricle Physiology ◽

Pulmonary Vasodilators ◽

Mean Pulmonary Arterial Pressure ◽

Pulmonary Vasodilator ◽

Pulmonary Arterial ◽

Stage 1

AbstractIn children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.

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Flow profile characteristics in Fontan circulation are associated with the single ventricle dilation and function: principal component analysis study

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00686.2019 ◽

2020 ◽

Vol 318 (5) ◽

pp. H1032-H1040 ◽

Cited By ~ 1

Author(s):

Michal Schäfer ◽

Benjamin S. Frank ◽

Stephen M. Humphries ◽

Kendall S. Hunter ◽

Katherine L. Carmody ◽

...

Keyword(s):

Single Ventricle ◽

Principal Component ◽

Ventricular Volume ◽

Fontan Circulation ◽

Collateral Flow ◽

Right Pulmonary Artery ◽

Flow Through ◽

The Right ◽

And Function ◽

Flow Waveforms

The Fontan circulation is characterized as a nonpulsatile flow propagation without a pressure-generating ventricle. However, flow through the Fontan circulation still exhibits oscillatory waves as a result of pressure changes generated by the systemic single ventricle. Identification of discrete flow patterns through the Fontan circuit may be important to understand single ventricle performance. Ninety-seven patients with Fontan circulation underwent phase-contrast MRI of the right pulmonary artery, yielding subject-specific flow waveforms. Principal component (PC) analysis was performed on preprocessed flow waveforms. Principal components were then correlated with standard MRI indices of function, volume, and aortopulmonary collateral flow. The first principal component (PC) described systolic versus diastolic-dominant flow through the Fontan circulation, accounting for 31.3% of the variance in all waveforms. The first PC correlated with end-diastolic volume ( R = 0.34, P = 0.001), and end-systolic volume ( R = 0.30, P = 0.003), cardiac index ( R = 0.51, P < 0.001), and the amount of aortopulmonary collateral flow ( R = 0.25, P = 0.027)—lower ventricular volumes and a smaller volume of collateral flow—were associated with diastolic-dominant cavopulmonary flow. The second PC accounted for 19.5% of variance and described late diastolic acceleration versus deceleration and correlated with ejection fraction—diastolic deceleration was associated with higher ejection fraction. Principal components describing the diastolic flow variations in pulmonary arteries are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to preserved ventricular volume and function, respectively. NEW & NOTEWORTHY The exact physiological significance of flow oscillations of phasic and temporal flow variations in Fontan circulation is unknown. With the use of principal component analysis, we discovered that flow variations in the right pulmonary artery of Fontan patients are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to more ideal ventricular volume and systolic function, respectively.

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Characterization of Extracorporeal Membrane Oxygenation Support for Single Ventricle Patients

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135119894294 ◽

2020 ◽

Vol 11 (2) ◽

pp. 183-191

Author(s):

Elizabeth H. Stephens ◽

Aqsa Shakoor ◽

Shimon E. Jacobs ◽

Shunpei Okochi ◽

Ariela L. Zenilman ◽

...

Keyword(s):

Extracorporeal Membrane Oxygenation ◽

Single Ventricle ◽

Early Recognition ◽

Orthotopic Heart Transplantation ◽

Factors Associated ◽

Membrane Oxygenation ◽

End Point ◽

Thrombotic Complications ◽

Central Cannulation

Background: Extracorporeal membrane oxygenation (ECMO) can provide crucial support for single ventricle (SV) patients at various stages of palliation. However, characterization of the utilization and outcomes of ECMO in these unique patients remains incompletely studied. Methods: We performed a single-center retrospective review of SV patients between 2010 and 2017 who underwent ECMO cannulation with primary end point of survival to discharge and secondary end point of survival to decannulation or orthotopic heart transplantation (OHT). Multivariate analysis was performed for factors predictive of survival to discharge and survival to decannulation. Results: Forty SV patients with a median age of one month (range: 3 days to 15 years) received ECMO support. The incidence of ECMO was 14% for stage I, 3% for stage II, and 4% for stage III. Twenty-seven (68%) patients survived to decannulation, and 21 (53%) patients survived to discharge, with seven survivors to discharge undergoing OHT. Complications included infection (40%), bleeding (40%), thrombosis (33%), and radiographic stroke (45%). Factors associated with survival to decannulation included pre-ECMO lactate (hazard ratio [HR]: 0.61, 95% confidence interval [CI]: 0.41-0.90, P = .013) and post-ECMO bicarbonate (HR: 1.24, 95% CI: 1.0-1.5, P = .018). Factors associated with survival to discharge included central cannulation (HR: 40.0, 95% CI: 3.1-500.0, P = .005) and lack of thrombotic complications (HR: 28.7, 95% CI: 2.1-382.9, P = .011). Conclusions: Extracorporeal membrane oxygenation can be useful to rescue SV patients with approximately half surviving to discharge, although complications are frequent. Early recognition of the role of heart transplant is imperative. Further study is required to identify areas for improvement in this population.

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