Cardiac catheterization in pulmonary hypertension: doing it right, with a catheter on the left

Hansmann, Georg; Rich, Stuart; Maron, Bradley A.

doi:10.21037/cdt-20-483

Cited by 5 publications

(4 citation statements)

References 31 publications

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“…Regarding the clinical course, the transition from PICU to ICM is much easier with an oral prostacyclin agonist (selexipag) than with IV treprostinil. Furthermore, we already had positive experiences regarding oral selexipag therapy in pediatric patients with severe PAH (17). In case of further dependence on mechanical ventilation, the ultima ratio would have been the listing for bilateral lung transplantion with an uncertain outcome considering prematurity, young age, and poor general condition (dystrophy).…”

Section: Discussionmentioning

confidence: 99%

“…A total of 3 months after PICU discharge and initiation of triple combination antihypertensive PH-targeted drug therapy, we performed right-left heart catheterization (RLHC) (Figure 2) at the age of 9 months. RLHC is essential for an accurate diagnosis of PH (8,17,18) and for new findings regarding relevant primary or secondary pathologies. Pressures derived from RLHC were as follows (mmHg): mean right atrial pressure (mRAP): 6; RV systolic/diastolic pressure: 34/6; pulmonary arterial pressure (PAP) systolic/diastolic: 29/17; mean PAP (mPAP): 23; systemic arterial pressure (SAP) systolic/diastolic: 76/47; mean SAP (mSAP): 61; pulmonary artery wedge pressure (PAWP): 10-12.…”

Section: Right-left-heart-catheterizationmentioning

confidence: 99%

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Case report: Rescue treatment with add-on selexipag in a preterm infant with suprasystemic pulmonary hypertension, pulmonary capillary hemangiomatosis, and isolated pulmonary vein stenosis

Hasan

Hysko

Jack

et al. 2022

Front. Cardiovasc. Med.

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An extremely dystrophic, premature female infant, born at 25 3/7 weeks of gestational age (birth weight: 430 g) with severe pulmonary hypertension (PH), was admitted to our neonatal intensive care unit (ICU) requiring cardiorespiratory support, including mechanical ventilation and pulmonary vasodilators such as inhaled nitric oxide (iNO) and continuous intravenous sildenafil infusions. The diagnosis of bronchopulmonary dysplasia (BPD) was made. A hemodynamically relevant, persistent ductus arteriosus (PDA) was surgically ligated after failed pharmacologic PDA closure using indomethacin and ibuprofen. The patient was discharged with an estimated 2/3 systemic pulmonary artery pressure. One month after hospital discharge, on low-flow oxygen supplementation (0.5 L/min FiO2 100%), at the corrected age of 16 weeks, she was readmitted to our emergency department with signs of respiratory distress and circulatory decompensation. Echocardiography demonstrated suprasystemic PH. Severe PH persisted despite initiated invasive mechanical ventilation, triple vasodilating therapy [iNO, macitentan, and continuous intravenous (IV) sildenafil], as well as levosimendan, milrinone, and norepinephrine for recompensation from cardiac shock. Thus, we started off-label oral selexipag therapy (oral IP receptor agonist) in the smallest patient reported so far (4 kg body weight). Subsequently, RV systolic pressure decreased to half-systemic, allowing successful weaning of iNO, norepinephrine, and milrinone, and extubation of the patient over 4 days. The infant was discharged 4 weeks after pediatric intensive care unit (PICU) admission in stable cardiorespiratory condition, with an oral, specific, triple antihypertensive PAH-targeted therapy using selexipag, macitentan, and sildenafil as well as oxygen therapy at low-flow (0.5 l/min) and spironolactone. The first cardiac catheterization at the age of 9 months under aforementioned triple PAH-targeted therapy revealed mild PH with 35% systemic PA pressure (mPAP/mSAP = 0.35) and isolated pulmonary vein stenosis. A transthoracic biopsy at the age of 12 months confirmed the diagnosis of BPD and further showed pulmonary interstitial glycogenosis and severe pulmonary capillary hemangiomatosis, without involvement of the pulmonary venules (chILD A2, A3, and B4 according to the Deutsch-Classification). The patient is currently in stable cardiorespiratory condition undergoing triple PH-targeted therapy including selexipag. This report highlights the potential benefits of the oral prostacyclin mimetic selexipag as an early add-on PH-targeted drug in chronic PH of infancy (cPHi).

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Section: Discussionmentioning

confidence: 99%

Section: Right-left-heart-catheterizationmentioning

confidence: 99%

Case report: Rescue treatment with add-on selexipag in a preterm infant with suprasystemic pulmonary hypertension, pulmonary capillary hemangiomatosis, and isolated pulmonary vein stenosis

Hasan

Hysko

Jack

et al. 2022

Front. Cardiovasc. Med.

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“…Postcapillary PH secondary to left heart disease can be isolated or (quite frequently) combined with a precapillary PH component ( Table 1 ) [ 1 , 2 ]. The details and caveats of the hemodynamic measurements in the catheterization laboratory, including the need to perform right and left heart catheterization, especially in PAH-CHD and in pediatric and adult patients with a suspected postcapillary component of PH, is discussed elsewhere [ 15 ].…”

Section: Introductionmentioning

confidence: 99%

Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

Meinel

Koestenberger

Sallmon³

et al. 2020

Diagnostics

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While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD.

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“…Coronary angiography may be indicated in selected cases when PH is confirmed. The authors suggest that overall, future consensus statements are needed that consider strengthening the indication for left heart catheterization in the diagnostic evaluation of PH patients by clarifying specific scenarios for which this approach is warranted (23).…”

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confidence: 99%

Getting to the bottom of right heart failure

Köestenberger

Bogaard

Hansmann

2020

Cardiovasc Diagn Ther

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Cardiac catheterization in pulmonary hypertension: doing it right, with a catheter on the left

Cited by 5 publications

References 31 publications

Case report: Rescue treatment with add-on selexipag in a preterm infant with suprasystemic pulmonary hypertension, pulmonary capillary hemangiomatosis, and isolated pulmonary vein stenosis

Case report: Rescue treatment with add-on selexipag in a preterm infant with suprasystemic pulmonary hypertension, pulmonary capillary hemangiomatosis, and isolated pulmonary vein stenosis

Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

Getting to the bottom of right heart failure

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