Aerosolized iloprost for postoperative pulmonary hypertensive crisis in children with congenital heart disease

Aims Pulmonary arterial hypertension is a severe complication in patients with congenital heart disease and poses a significant risk to women wishing to become pregnant. This study describes the clinical presentation, maternal outcomes and risk factors for the peripartum period in women with pulmonary arterial hypertension related to congenital heart disease (PAH-CHD). Methods All pregnant women with PAH-CHD who were admitted for delivery in a tertiary center between February 2011–September 2016 were included. Logistic regression analysis was used to identify predictors of the combined endpoint of maternal death, severe heart failure requiring treatment, or pulmonary hypertensive crisis. Results Ninety-three women (94 pregnancies) were included. Average age was 27.5 ± 4.4 years. Thirty (31.9%) patients had Eisenmenger syndrome, 51 (54.3%) had pulmonary arterial hypertension associated with systemic-to-pulmonary shunts, and 13 (13.8%) had pulmonary arterial hypertension with corrected congenital heart disease. Twenty-three (24.5%) women required admission for delivery within two days from presentation. Elective Cesarean section was performed in 95.7% of women, with intravertebral anesthesia in 93.6%. Fifty-one (54.2%) patients received pulmonary arterial hypertension therapies during pregnancy. Six (6.4%) women died, 33 (35.1%) developed heart failure and 10 (10.6%) had a pulmonary hypertensive crisis. Patients who met the combined endpoint ( n = 34, 36.2%) were more likely to have Eisenmenger syndrome or repaired defects ( p < 0.001). Other risk factors in the multivariate model included lower arterial blood oxygen saturation, higher brain natriuretic peptide, and pericardial effusion on echocardiography. Conclusion Maternal mortality and morbidity remain high in PAH-CHD patients, who should be counseled on the risks of pregnancy and managed in a tertiary multidisciplinary environment to improve prognosis.

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Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-6.3.121 ◽

2007 ◽

Vol 6 (3) ◽

pp. 121-125 ◽

Cited By ~ 1

Author(s):

Ingram Schulze-Neick ◽

John E. Deanfield

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Congenital Heart ◽

Vascular System ◽

Hypertensive Crisis ◽

Multidisciplinary Care ◽

Structural Heart Disease ◽

Pulmonary Arterial

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

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Monitored Anesthesia Care of Two Patients with Highly Elevated Subpulmonic Ventricular Pressure due to Adult Congenital Heart Disease

Case Reports in Cardiology ◽

10.1155/2020/2040561 ◽

2020 ◽

Vol 2020 ◽

pp. 1-6

Author(s):

Tatsuya Kida ◽

Tomoya Irie ◽

Takahisa Goto

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Respiratory Depression ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

Hypertensive Crisis ◽

Ventricular Pressure ◽

Anesthesia Care ◽

Monitored Anesthesia Care ◽

Adult Congenital

Procedural sedation and analgesia for patients with adult congenital heart disease (ACHD) and highly elevated subpulmonic ventricular pressure require proper anesthesia care to prevent a pulmonary hypertensive crisis. We describe the monitored anesthesia care (MAC) of two patients with ACHD (a complete atrioventricular septal defect and congenitally corrected transposition of the great arteries) and highly elevated subpulmonic ventricular pressure. In both patients, preprocedural transthoracic echocardiography was useful for detecting severely elevated subpulmonic ventricular pressure. The MAC involved the infusion of propofol, dexmedetomidine, and fentanyl. Norepinephrine was continuously administered from the preanesthetic period. No hemodynamic instability or respiratory depression was observed during the MAC. Continuous administration of norepinephrine from the preinduction period was helpful for preventing hypotension. We added dexmedetomidine to our MAC regimen of propofol and fentanyl because it exerts both sedative and analgesic effects. Dexmedetomidine does not cause respiratory depression; thus, our MAC regimen is believed to be theoretically safe for patients with ACHD and elevated subpulmonic ventricular pressure. Our findings suggest that safe MAC for patients with ACHD and highly elevated subpulmonic ventricular pressure may require careful consideration of the anesthetic regimen and close observation by adequately trained personnel, which is best provided at regional ACHD centers.

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Pheochromocytoma in Congenital Cyanotic Heart Disease

Case Reports in Endocrinology ◽

10.1155/2018/2091257 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Carmen Aresta ◽

Gianfranco Butera ◽

Antonietta Tufano ◽

Giorgia Grassi ◽

Livio Luzi ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Histopathological Examination ◽

Hypertensive Crisis ◽

Genetic Alterations ◽

Molecular Response ◽

Catecholamine Excess ◽

Brown Adipose ◽

Increased Risk

Studies on genome-wide transcription patterns have shown that many genetic alterations implicated in pheochromocytoma-paraganglioma (P-PGL) syndromes cluster in a common cellular pathway leading to aberrant activation of molecular response to hypoxia in normoxic conditions (the pseudohypoxia hypothesis). Several cases of P-PGL have been reported in patients with cyanotic congenital heart disease (CCHD). Patients affected with CCHD have an increased likelihood of P-PGL compared to those affected with noncyanotic congenital heart disease. One widely supported hypothesis is that chronic hypoxia represents the determining factor supporting this increased risk. We report the case of a 23-year-old woman affected with congenital tricuspid atresia surgically by the Fontan procedure. The patient was admitted to hospital with hypertensive crisis and dyspnea. Chest computed tomography revealed, incidentally, a 6-cm mass in the left adrenal lodge. Increased levels of noradrenaline (NA) and its metabolites were detected (plasma NA 5003.7 pg/ml, n.v.<480; urinary NA 1059.5 µg/24 h, n.v.<85.5; urinary metanephrine 489 µg/24 h, n.v.<320). The patient did not report any additional symptom related to catecholamine excess. The left adrenal tumor showed abnormal accumulation when 131I-metaiodobenzylguanidine scintigraphy was performed. A 18F-fluorodeoxyglucose positron emission tomography showed no significant metabolic activity in the left adrenal gland but intense uptake in the supra- and subdiaphragmatic brown adipose tissue, probably due to noradrenergic-stimulated glucose uptake. The patient underwent left open adrenalectomy after preconditioning with α- and β-blockers and histopathological examination confirmed the diagnosis of pheochromocytoma (Ki-67<5%). Screening for germline mutations did not show any genes mutation (investigated mutations: RET, TMEM127, MAX, SDHD, SDHC, SDHB, SDHAF2, SDHA, and VHL). Clinicians should consider P-PGL when an unexplained clinical deterioration occurs in CCHD patients, even in the absence of typical paroxysmal symptoms.

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