Potts shunt in children with pulmonary arterial hypertension: institutional experience

Горбачевский, С.В.; Шмальц, А.А.; Barishnikova, Irina Y; Zaets, Sergey B.

doi:10.1093/icvts/ivx209

Cited by 12 publications

(4 citation statements)

References 21 publications

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“…Based on the research accomplished by Gorbachevsky et al [26], while a Potts shunt can dramatically minimize the chances of syncope, hypoxemia of the lower limbs is moderate and highly endurable. They also showed that a Potts shunt could decrease the right ventricle-to-left ventricle end-diastolic diameter ratio, per echocardiography, and the suprasystemic pulmonary hypertension is decreased to the systemic level, which ultimately equalizes the pressure of both ventricles [26].…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

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

2021

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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.

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Section: Discussion and Review Of The Literaturementioning

confidence: 99%

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

2021

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“…Potts shunt has already been shown as being a durable palliative approach for the management of patients who aggravate despite maximal medical treatment 9–12 . In addition, it may also be indicated in patients who have recurrent syncopes particularly at exercise while having infrasystemic resting pulmonary artery pressure 13,14 .…”

Section: Discussionmentioning

confidence: 99%

Potts anastomosis in children with severe pulmonary arterial hypertension and atrial septal defect

et al. 2020

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Aims Potts shunt has been proposed as a bridge or alternative to lung transplantation for children with severe and drug‐refractory suprasystemic pulmonary arterial hypertension (PAH). We describe the management of the atrial shunt when a Potts shunt is planned in refractory PAH. Methods and results We report a case series of children in whom a Potts shunt was done for severe PAH associated with an atrial septal defect to illustrate the different clinical and haemodynamic scenarios. Five children (2 to 13 years) underwent a Potts shunt: three surgical, one percutaneous Potts shunt, and one percutaneous stenting of a restrictive arterial duct. All had associated atrial septal defect. Those who had generalized cyanosis before the procedure had a complicated postoperative course and required longer ventilatory and inotropic support, except the one who had atrial septal defect closure before the Potts shunt. One of the three cyanotic patients died. Two patients with left‐to‐right shunt before the Potts shunt had an uncomplicated postoperative course. Conclusions Shunt physiology is only partially predictable after the Potts shunt in children with PAH and atrial septal defect. Abrupt drop in left ventricle preload while the right ventricle is decompressed can potentially be prevented by atrial septal defect closure prior to the Potts shunt.

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“…Bei kleineren Kindern konnte ein verbessertes Gedeihen gezeigt werden. Am beeindruckendsten ist, dass in zahlreichen Fällen auch die medikamentöse Therapie mit systemischen Prostazyklinen beendet werden konnte [56][57][58]. Die kürzlich publizierte internationale Potts-Registerstudie fasst die Langzeitergebnisse von insgesamt 110 Kindern zusammen.…”

Section: Risiko Und Langzeitverlaufunclassified

Interventionelle Behandlungsverfahren der pulmonalen Hypertension im Kindesalter

Albinni

Kitzmüller

Beichl

et al. 2022

Monatsschr Kinderheilkd

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ZusammenfassungDie pulmonale Hypertension (PH) ist eine progressive Gefäßerkrankung und führt über eine Widerstands- und/oder Druckerhöhung im kleinen Kreislauf zu einem fortschreitenden Rechtsherzversagen. Auch wenn mithilfe aktueller medikamentöser Therapien eine deutliche Verbesserung der Lebensqualität und des Überlebens der Betroffenen erreicht werden konnte, bleibt die PH eine zumeist nichtheilbare Erkrankung, die im fortgeschrittenen Stadium eine Lungentransplantation notwendig macht. Interventionelle Verfahren, wie die Anlage eines interatrialen Shunts (z. B. durch atriale Septostomie oder den Atrial Flow Regulator) oder eines Reversed Potts Shunt, verbessern die RV-Funktion und die ventrikuläre Interaktion durch Schaffung einer prä- oder posttrikuspidalen „Eisenmenger-Physiologie“ und stellen eine Überbrückung oder sogar Alternative zur Lungentransplantation dar. Bei Patienten mit segmentaler PH oder chronisch thrombembolischer pulmonaler Hypertension (CTEPH) stellt die Ballonangioplastie eine bereits etablierte Intervention zur Verbesserung der pulmonalen Perfusion und damit rechtsventrikulären (RV-)Funktion dar. Dagegen ist die pulmonalarterielle Denervation ein neuartiges Verfahren, mit dem Ziel, die neurohumorale Dysregulation bei PH positiv zu beeinflussen. Der individuelle Einsatz solcher Interventionen, additiv zu den bereits etablierten medikamentösen Therapien, erweitert die Behandlungsmöglichkeiten und kann die Prognose betroffener Patienten noch weiter verbessern.

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Potts shunt in children with pulmonary arterial hypertension: institutional experience

Abstract: Our limited experience demonstrates that the Potts shunt lowers suprasystemic pulmonary hypertension to the systemic level, limits manifestations of right-sided heart failure and improves the overall functional status of the patients.

Cited by 12 publications

References 21 publications

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

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

Potts anastomosis in children with severe pulmonary arterial hypertension and atrial septal defect

Interventionelle Behandlungsverfahren der pulmonalen Hypertension im Kindesalter

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