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6 results

  • Chapter 43 - Pulmonary Hypertension and Moyamoya Disease

  • from Section 7 - Miscellaneous Lesions and Syndromes
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 331-337

  • Summary

    Moyamoya disease is a cerebrovasculopathy of unknown etiology characterized by chronic progressive stenosis of the arteries of the circle of Willis. An extensive collateralized circulation forms, giving rise to the smoky appearance seen on cerebral angiography (moyamoya is Japanese for “puff of smoke”). Primary clinical features of moyamoya disease, especially in children, are related to cerebral ischemia and include transient ischemic attacks and stroke. The goals of treatment are primarily to restore blood flow to the affected areas, either by direct or indirect bypass, as there is no curative treatment for moyamoya disease. This chapter details the perioperative management of a patient with coexisting morbidities of moyamoya disease, coarctation of the aorta, and pulmonary hypertension, discussing the concerns related to each and their considerations in developing an anesthetic management plan.

  • The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation

  • Ahmed Krimly, C. Charles Jain, Alexander Egbe, Ahmed Alzahrani, Khalid Al Najashi, Dimpna Albert-Brotons, Gruschen R. Veldtman
  • Journal:
    Cardiology in the Young / Volume 31 / Issue 8 / August 2021
    Published online by Cambridge University Press:
    11 August 2021, pp. 1241-1250

  • Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

  • Acquired and progressive coronary arterial fistulae in patients with single-ventricle physiology and treated with pulmonary vasodilators

  • Part of
  • Kanta Kishi, Hiroshi Katayama, Shintaro Nemoto, Noriyasu Ozaki, Yutaka Odanaka, Atsuko Ashida, Hayato Konishi, Akira Ashida
  • Journal:
    Cardiology in the Young / Volume 31 / Issue 11 / November 2021
    Published online by Cambridge University Press:
    22 March 2021, pp. 1823-1828
  • Background:

    Cardiac dysfunction, arrhythmia, and hepatic fibrosis are well-known complications after right heart bypass surgery in patients with single-ventricle physiology. However, little is known about coronary arterial fistulae, and only a few reports have been published. This study aimed to elucidate the clinical characteristics of these rare coronary arterial fistulae that developed as complications in cases of single-ventricle physiology after right heart bypass surgery.

    Methods:

    We retrospectively investigated the clinical features and courses of patients who developed acquired and progressive coronary arterial fistulae after right heart bypass surgery in our hospital.

    Results:

    We identified three cases of coronary arterial fistulae out of 21 patients who underwent right heart bypass surgery. All three cases underwent cardiac catheterisation for post-operative evaluation and were administered pulmonary vasodilators of phosphodiesterase type V inhibitors, antiplatelet, anticoagulation, and diuretics. Moreover, they had common clinical features such as right-dominant single ventricle and long-term exposure to chronic hypoxia. Serial angiograms revealed acquired and progressive coronary arterial fistulae. In addition, coronary arterial fistulae contributed to their symptoms of heart failure.

    Conclusion:

    Patients with chronic hypoxia and dominant right ventricle, who are treated with phosphodiesterase type V inhibitors, should be followed up after right heart bypass surgery to monitor the possible development of coronary arterial fistulae. Moreover, the indication for pulmonary vasodilators in single-ventricle physiology after right heart bypass surgery should be optimised to avoid adverse effects.

  • Pulmonary vasodilator therapy and early postoperative outcome after modified Fontan operation

  • Alberto Mendoza, Leticia Albert, Sylvia Belda, Lidia Casanueva, Dolores Herrera, Miguel A. Granados, José M. Velasco, Enrique García, Juan M. Aguilar, Juan V. Comas
  • Journal:
    Cardiology in the Young / Volume 25 / Issue 6 / August 2015
    Published online by Cambridge University Press:
    16 September 2014, pp. 1136-1140

  • Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome.

    A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009.

    Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.