Progressive pulmonary vascular disease after surgery in a case of patent ductus arteriosus with pulmonay hypertension.

Yamaki, Shohei; Ishidoya, Takeshi; Osuga, Yo; Arai, Satoru

doi:10.1620/tjem.140.279

Cited by 10 publications

(2 citation statements)

References 15 publications

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“…42 Some infants and children with a large patent ductus do not experience the normal postnatal fall in pulmonary vascular resistance, and even after closure of the ductus, pulmonary vascular disease may progress and eventually prove fatal. 42,43 …”

Section: Hypertensive Pulmonary Vascular Diseasementioning

confidence: 99%

Patent Ductus Arteriosus

2006

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T he patent ductus arteriosus (PDA) is a vascular structure that connects the proximal descending aorta to the roof of the main pulmonary artery near the origin of the left branch pulmonary artery. This essential fetal structure normally closes spontaneously after birth. After the first few weeks of life, persistence of ductal patency is abnormal. The physiological impact and clinical significance of the PDA depend largely on its size and the underlying cardiovascular status of the patient. The PDA may be "silent" (not evident clinically but diagnosed incidentally by echocardiography done for a different reason), small, moderate, or large. Regardless of the size, complications may arise, and it is important for both pediatric and adult cardiologists to have an understanding of the pathophysiology, clinical implications, and management of PDA. EmbryologyThe ductus arteriosus is a normal and essential fetal structure that becomes abnormal if it remains patent after the neonatal period. In normal cardiovascular development, the proximal portions of the sixth pair of embryonic aortic arches persist as the proximal branch pulmonary arteries, and the distal portion of the left sixth arch persists as the ductus arteriosus, connecting the left pulmonary artery with the left dorsal aorta (Figure 1). Normally, the distal right sixth aortic arch loses its connection to the dorsal aorta and degenerates. This transformation is complete by 8 weeks of fetal life. Normal Physiology Fetal CirculationWhereas Ϸ65% of the fetal cardiac output is from the right ventricle, only 5% to 10% passes through the lungs. 1,2 The preponderance of right ventricular output passes through the ductus arteriosus into the descending aorta. The fetal ductus arteriosus is thus an important structure that is essential for normal fetal development, permitting right ventricular output to be diverted away from the high-resistance pulmonary circulation. Premature constriction or closure may lead to right heart failure, resulting in fetal hydrops. 3 Histology and Mechanisms of Normal ClosureGrossly, the constitution of the fetal ductus arteriosus appears to be similar to the contiguous main pulmonary artery and descending aorta; there are important histological differences, however. 4 -9 Whereas the mediae of surrounding aorta and pulmonary artery are composed mainly of circumferentially arranged layers of elastic fibers, the media of the ductus arteriosus is composed of longitudinally and spirally arranged layers of smooth muscle fibers within loose, concentric layers of elastic tissue. The intima of the ductus arteriosus is thickened and irregular, with abundant mucoid material, sometimes referred to as intimal cushions.Fetal patency of the ductus arteriosus is controlled by many factors, the most important of which are relatively low fetal oxygen tension 10 and cyclooxygenase-mediated products of arachidonic acid metabolism (primarily prostaglandin [PGE 2 ] and prostacyclin [PGI 2 ]). 11 Locally produced and circulating PGE 2 and PGI 2 in the fetus cau...

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Section: Hypertensive Pulmonary Vascular Diseasementioning

confidence: 99%

Patent Ductus Arteriosus

2006

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“…Not only is there the risk of peri-operative mortality, the likelihood of right heart failure from progressive increase in PVR despite repair of the defects exists. [18] In practice, the quality of life of such patients is far worse than that of patients with Eisenmenger syndrome and PDA followed up medically. Therefore it is important to be certain of operability before referring such patients for surgery.…”

Section: Pulmonary Hypertension and Pulmonary Vascular Disease (Pvd) mentioning

confidence: 99%