J Raine

Bio: J Raine is an academic researcher. The author has contributed to research in topics: Lumen (anatomy) & Breathing. The author has an hindex of 3, co-authored 3 publications receiving 72 citations.

Tetralogy of Fallot with anomalous pulmonary venous connections: a rare but clinically important association.

Abstract: Anomalous pulmonary venous connections were found in seven (0.6%) of 1183 patients with tetralogy of Fallot. Three patients had totally anomalous connections (one supracardiac, one direct to coronary sinus, and one mixed supracardiac and infracardiac) and four patients had partially anomalous pulmonary venous connections. All patients presented with the clinical features of tetralogy of Fallot. Anomalous pulmonary venous drainage was suspected clinically in only one patient in whom there was a scimitar sign on the chest radiograph. The exact diagnosis was established by cross sectional echocardiography (one), preoperative or postoperative angiography (five), or at necropsy (one). Surgery was performed in six patients. Total correction without re-routing of the anomalously draining veins was successful in all those with partially anomalous connections, with no significant long term sequelae (follow up median 17 years). Of those with totally anomalous connections, the full diagnosis was made only at necropsy in one patient, successful one-stage correction was performed in one, and the other patient, who had partially obstructed mixed drainage, died shortly after one-stage correction. Histological examination of the lung biopsy specimen in this patient showed grade 2 pulmonary vascular disease. Tetralogy of Fallot with anomalous pulmonary venous connections is a rare association. Careful preoperative assessment is required in those with totally anomalous connections.

Negative extrathoracic pressure ventilation for phrenic nerve palsy after paediatric cardiac surgery.

Abstract: OBJECTIVE--To investigate the feasibility of negative extrathoracic pressure ventilation as a respiratory support following phrenic nerve palsy after cardiac surgery. DESIGN--An uncontrolled pilot study. PATIENTS--14 patients aged one week to 30 months (median 5.3 months) with phrenic nerve palsy diagnosed by phrenic nerve conduction tests and diaphragmatic electromyograms. Four had bilateral and 10 unilateral palsy. Before treatment all required oxygen and 10 were receiving positive pressure ventilation. One of the patients with bilateral and four of the patients with unilateral palsies had undergone a plication before negative pressure ventilation was started. INTERVENTION--Treatment was started 6-65 days (median 23) after operation with a newly designed system which included a Perspex chamber, which gave easy access to the child, and an elastic latex neck seal. Continuous negative pressure was used in conjunction with intermittent positive pressure ventilation while continuous or intermittent negative pressure ventilation was used in extubated infants. RESULTS--All four patients with bilateral palsy survived with long-term intermittent negative pressure ventilation and did not require further surgery. Of the 10 with unilateral lesions, seven required no further surgery, two underwent plication, and one had a re-plication. Three patients with unilateral palsy died of non-respiratory causes. The duration of positive pressure ventilation after starting negative pressure ranged from 0 to 23 days (median 6). Treatment with negative pressure lasted for 3-241 days (median 32) and was predominantly administered off the intensive care unit, including at home. CONCLUSIONS--Negative pressure ventilation may be an alternative to positive airway pressure ventilation in the management of phrenic nerve palsy. A multicentre randomised controlled trial is now required to assess further the role of negative pressure ventilation in phrenic nerve palsy.

Fatal persistent pulmonary hypertension presenting late in the neonatal period.

Abstract: Two cases of fatal idiopathic persistent pulmonary hypertension presented late in the neonatal period. Lungs were examined histologically by light and electron microscopy, and immunocytochemical studies were used to identify nerves. There was extension of medial smooth muscle distally along the arterial pathway so that most precapillary arteries had completely muscular walls, which in some cases completely obliterated the vessel lumen. Enlarged endothelial cells also contributed to the reduction in the size of the lumen. Nerve fibres accompanying muscular arteries were found in the alveolar region, more distal than is normal. The predominant neuropeptide was the vasoconstrictor tyrosine. Possible aetiological factors in persistent pulmonary hypertension of the newborn are increased muscularity of the peripheral pulmonary arteries antenatally, an increase in the number of vasoconstrictor nerves, or an imbalance in the production of leukotrienes and prostacyclins in the perinatal period.

Pulmonary arterial hypertension in children.

Abstract: Pulmonary arterial hypertension is a serious progressive condition with a poor prognosis if not identified and treated early. Because the symptoms are nonspecific and the physical findings can be subtle, the disease is often diagnosed in its later stages. Remarkable progress has been made in the field of pulmonary arterial hypertension over the past several decades. The pathology is now better defined, and significant advances have occurred in understanding the pathobiologic mechanisms. Risk factors have been identified, and the genetics have been characterized. Advances in technology allow earlier diagnosis as well as better assessment of disease severity. Therapeutic modalities such as new drugs, e.g., epoprostenol, treprostinil, and bosentan, and surgical/interventional options, e.g., transplantation and atrial septostomy, which were unavailable several decades ago, have had a significant impact on prognosis and outcome. Thus, despite our inability to cure pulmonary arterial hypertension, advances in medical treatments over the past two decades have resulted in significant improvement in outcomes for children with various forms of pulmonary arterial hypertension. This report is a review the current state of the art for pulmonary arterial hypertension in 2004, with an emphasis on childhood pulmonary arterial hypertension and specific recommendations for current practice and future directions.

Chronic in ovo hypoxia decreases pulmonary arterial contractile reactivity and induces biventricular cardiac enlargement in the chicken embryo

Abstract: Although chronic prenatal hypoxia is considered a major cause of persistent pulmonary hypertension of the newborn, experimental studies have failed to consistently find pulmonary hypertensive changes after chronic intrauterine hypoxia. We hypothesized that chronic prenatal hypoxia induces changes in the pulmonary vasculature of the chicken embryo. We analyzed pulmonary arterial reactivity and structure and heart morphology of chicken embryos maintained from days 6 to 19 of the 21-day incubation period under normoxic (21% O(2)) or hypoxic (15% O(2)) conditions. Hypoxia increased mortality (0.46 vs. 0.14; P < 0.01) and reduced the body mass of the surviving 19-day embryos (22.4 +/- 0.5 vs. 26.6 +/- 0.7 g; P < 0.01). A decrease in the response of the pulmonary artery to KCl was observed in the 19-day hypoxic embryos. The contractile responses to endothelin-1, the thromboxane A(2) mimetic U-46619, norepinephrine, and electrical-field stimulation were also reduced in a proportion similar to that observed for KCl-induced contractions. In contrast, no hypoxia-induced decrease of response to vasoconstrictors was observed in externally pipped 21-day embryos (incubated under normoxia for the last 2 days). Relaxations induced by ACh, sodium nitroprusside, or forskolin were unaffected by chronic hypoxia in the pulmonary artery, but femoral artery segments of 19-day hypoxic embryos were significantly less sensitive to ACh than arteries of control embryos [pD(2) (= -log EC(50)): 6.51 +/- 0.1 vs. 7.05 +/- 0.1, P < 0.01]. Pulmonary vessel density, percent wall area, and periarterial sympathetic nerve density were not different between control and hypoxic embryos. In contrast, hypoxic hearts showed an increase in right and left ventricular wall area and thickness. We conclude that, in the chicken embryo, chronic moderate hypoxia during incubation transiently reduced pulmonary arterial contractile reactivity, impaired endothelium-dependent relaxation of femoral but not pulmonary arteries, and induced biventricular cardiac hypertrophy.

Negative pressure ventilation in the treatment of acute respiratory failure: an old noninvasive technique reconsidered

Abstract: Noninvasive mechanical ventilatory techniques include the use of negative and positive pressure ventilators. Negative pressure ventilators, such as the "iron lung", support ventilation by exposing the surface of the chest wall to subatmospheric pressure during inspiration; whereas, expiration occurs when the pressure around the chest wall increases and becomes atmospheric or greater than atmospheric. In this review, after a description of the more advanced models of tank ventilators and the physiological effects of negative pressure ventilation (NPV), we summarize the recent application of this old technique in the treatment of acute respiratory failure (ARF). Several uncontrolled studies suggest that NPV may have a potential therapeutic role in the treatment of acute on chronic respiratory failure in patients with chronic obstructive pulmonary disease and restrictive thoracic disorders, reducing the need for endotracheal intubation. In the paediatric field, after substantial technical improvement, NPV has been successfully reintroduced for the treatment of ARF due to neonatal distress syndrome and bronchopulmonary dysplasia, and for the weaning from positive pressure ventilation in intubated patients. The positive results of these reports need to be formally confirmed by further prospective and controlled studies before recommending the generalized use of negative pressure ventilation in acute respiratory failure as a standard of care.