Isoflurane and primary pulmonary hypertension

Primary pulmonary hypertension is a rare and fatal disease. These patients represent an anaesthetic challenge because of the high mortality associated with the marked intra-operative increase in pulmonary vascular resistance and right ventricular decompensation. This is a first case report which demonstrates the safe and beneficial effects of isoflurane in lowering pulmonary arterial pressure and pulmonary vascular resistance in such a patient for a short surgical procedure. The anaesthetic considerations in these patients are discussed.     

Surgical treatment of chronic thrombo-embolic pulmonary hypertension with floating thrombi in the right heart ventricle

A 31 years old male patient was admitted to a resuscitation unit with severe dyspnea (MMRC 4 grade). The patient had the dyspnea for 3 months.The diagnosis of chronic thrombo-embolic pulmonary hypertension with floating thrombi in the right heart ventricle was established. On the sixth day of admission after IVC filter insertion, the patient underwent thromboectomy with pulmonary endarterectomy. For an access to segmentary pulmonary arteries during operation SVC and the aorta had been cross-clamped. The duration of deep hypothermic (20 degrees C) circulatory arrest was 54 minutes. The patient was discharged in satisfactory condition on the 17th day. At the check-up at 3 months the dyspnea was absent and heart hemodynamic parameters had been normalized.     

A successful case of pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle

Chronic thromboembolism is a frequent cause of progressive hypertension and carries a poor prognosis. Medical treatment is not effective and surgery provides the only potential for a cure at present. We herein report a successful case of thromboendarterectomy treated via a median sternotomy with intermittent circulatory arrest. A 43-year-old man was admitted to our hospital complaining of progressive dyspnea, edema of the lower extremities, and a fever with an unknown origin. A subsequent definitive evaluation showed him to be suffering from surgically accessible chronic thromboembolic pulmonary hypertension with a thrombus in the right ventricle. He underwent a pulmonary thromboen-darterectomy and thrombectomy via a median sternotomy with intermittent circulatory arrest on November 24, 1994. Postoperatively he showed a marked improvement in his hemodynamic status and blood gas analysis. He has also returned to work with no trouble. Deep vein thrombosis appeared to be the pathogenesis of this case, but we could not find the origin of his unknown fever. He is currently being controlled by treatment with methylprednisolone as before.     

Novel strategies for the management of right ventricular failure with pulmonary hypertension

The authors report a case where four novel strategies were successfully used in the management of advanced right ventricular failure with severe pulmonary hypertension and complex adult congenital heart disease. These included combination and optimisation of three advanced therapies for pulmonary hypertension, therapeutic abdominal paracentesis, correction of underlying metabolic/endocrine disturbance and a new inotropic agent.     

Circulatory support with right ventricular assist device and intra-aortic balloon counterpulsation in patient with right ventricle failure after pulmonary embolectomy

Severe pulmonary embolism may lead to acute right ventricular failure despite immediate surgical embolectomy, which is regarded as the treatment of choice after recent CABG surgery. We report a case of a patient with massive pulmonary thromboembolism which resulted in acute right ventricular failure following early surgical embolectomy. Pulmonary embolism developed two days after an elective off-pump CABG surgery. We observed severe circulatory collapse which resulted in cardiac arrest and proved refractory to pharmacological treatment after immediate cardiopulmonary resuscitation. Intra-aortic balloon pumping was used in an attempt to improve hemodynamic performance during surgical skin preparation. After the completion of the embolectomy and failure to wean the patient from CPB, upon clinical signs of low cardiac output and akinetic right ventricle, the decision was made to support its function with a centrifugal pump. The substantial improvement of the right ventricular function observed in the next 24 h allowed weaning the patient from right ventricle support. In spite of hemodynamic recovery, the patient remained in a coma on discharge from the cardiac-surgical ICU after 18 days, and died 10 days later from systemic infection.     

Origin of the aorta and pulmonary artery from the right ventricle

In the period of 1980 to February 1988, 60 patients aged 1 year 7 months to 23.5 years were operated on for concordant atrioventricular linkage and origin of the aorta and pulmonary artery from the right ventricle. Sixty-two heart specimens were examined to study the defect anatomy. The left ventricular outlet opened up in the subaortic cone (Type A defect) in 47 (78.3%) patients, in the common subaortic and subpulmonary cone (Type B) in 8 (13.4%) patients, in the subpulmonary cone (Type C) in 3 (5%) patients; 2 (3.3%) subjects exhibited nonconcomitant ventricular septal defect (Type D). Thirty-four (56%) patients had pulmonary stenosis. Concomitant heart diseases were found in 78%. Fifteen patients (25%) underwent radical operation after prior palliative interventions. The total fatal cases were 13.7% (out of 58 operated patients, 8 died) in the group of radically operated patients. The fatal outcomes were observed in Type A defect. The analysis showed that defect correction techniques, age, prior palliative interventions, presence or absence of pulmonary stenosis, concurrent heart diseases, and restrictive ventricular septal defect failed to affect the immediate result of surgeries. An inaccurate preoperative diagnosis and lack of surgical experiment were factors increasing the risk for correction from 1980 to 1986. In the 2 years, the death rates decreased from 17.9% (7 dead patients per 39 operated ones) to 5.3% (1 dead patient per 19 radically operated ones).     

Left ventricle is better suited as pulmonary ventricle in simple transposition with severe pulmonary hypertension

BACKGROUND: The conventional treatment of transposition of great arteries with prepared left ventricle is an arterial switch operation. This, in our experience, does not hold for patients with transposition of great arteries with intact ventricular septum where the left ventricle continues to be prepared secondary to severe pulmonary arterial hypertension without an immediately reversible cause. METHODS: Ten infants with D-transposition of the great arteries with essentially intact interventricular septum and severe pulmonary arterial hypertension underwent surgical treatment. Age ranged from 3 to 6 months (mean, 4.2 months). One of these patients had a large ductus with left to right shunting but the others had no intra- or extracardiac shunt to account for their pulmonary hypertension. All 10 had "prepared" left ventricles. The first 4 children underwent an arterial switch operation. Uneventful surgery was followed by prolonged ventilator dependence in all 4 with occurrence of severe pulmonary arterial hypertension every time weaning from ventilator was attempted. This was accompanied by metabolic acidosis and features of right heart failure. Only 1 patient with large ductus could be extubated and discharged from hospital. Subsequently, the other 6 infants underwent a Senning repair. RESULTS: There was no early mortality. All patients were separated from mechanical ventilation within 48 hours of surgery without blood gas derangement or heart failure despite elevated pulmonary artery pressure in all. The child with the arterial switch operation has pulmonary artery pressure of 50% systemic 4 years following repair; although among the Senning group, 2 patients continue to have pulmonary artery pressure more than 60% of systemic and 4 have normal pulmonary artery pressure at a mean follow-up of 1 year. CONCLUSIONS: Atrial level repairs seem to perform better than arterial level repairs in children having TGA with persistent pulmonary artery hypertension without a correctable cause. Better tolerance of pulmonary arterial hypertension in this group is probably consequent to the superior ability of the left ventricle to tolerate a pressure load in the early postoperative period.     

Life-threatening pulmonary hypertension and right ventricular failure complicating calcium and phosphate replacement in the intensive care unit

A 43-year-old man developed septic shock and acute lung injury after surgery to drain an ischiorectal abscess. In the intensive care unit he initially improved but developed severe hypoxaemia, right ventricular failure and pulmonary hypertension 90 min after receiving intravenous calcium gluconate and potassium phosphate, best explained by the formation of a calcium-phosphate precipitant that resulted in aggregate anaphylaxis. His rapid deterioration and lack of response to conventional therapies necessitated support with extracorporeal membrane oxygenation that was life saving. This adverse event has altered local practice regarding calcium and phosphate replacement and has implications for all intensive care units.     

Heart failure due to rapid right ventricular volume overload in patients with pulmonary hypertension

We have described three cases of pulmonary artery hypertension (PAH). Two of them developed severe systemic hypotension and cardiac arrest following cardiopulmonary bypass (CPB). Imminent pre-operative right ventricular (RV) failure and subsequent myocardial injury during CPB may have contributed to the insult following CPB. In these cases, RV failure is presumably due to rapid volume overloading, since left atrial pressure (LAP) was essentially unchanged while right atrial pressure (RAP) was markedly elevated during the period of severe hypotension. Furthermore, these events may be attributed to protamine, since the circulatory derangement coincides with its administration. Thus, in these patients with severe PAH, blood transfusion should be carefully titrated under the strict evaluation of both LAP and RAP. Additionally, protamine should be infused at a rate slow enough to avoid concomitant hemodynamic changes. We have also reported a case of over-systemic PAH who was successfully managed intraoperatively by careful monitoring and discreet administration of protamine.     

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure

We describe here successful palliative repair of tricuspid atresia, hypoplastic right ventricle, transposition of the great arteries, and hypoplastic aortic arch in a neonate. The repair consisted of the Norwood procedure with a rudimentary right ventricle to pulmonary artery shunt, which was located on the right side of a neo-aorta. This procedure could be a useful adjunct to avoid left ventriculotomy and its subsequent dysfunction.     

Classification of aortic and pulmonary artery origin from right ventricle

An original systematics of origin of the aorta and pulmonary artery from the right ventricle (OAPA RV) is suggested on the basis of examination of 63 heart specimens with the anomaly. Two main variants of OAPA RV are distinguished: (1) with and (2) without a formed infundibular septum (IS). Types A and DA belong to the first variant, types B, C, D, and DC to the second variant. Type A is characterized by drainage of the left ventricular (LV) outflow tract (OT) into the subaortic conus. Type DA differs from type A by the existence of an auxiliary opening between the ventricles due to a deficiency of tissue of the posterior (sinus part) of the septum. Type B is characterized by the absence (or marked hypoplasia) of the septum of the conus (SC). The LV OT in this case drains under both (aortic and pulmonary) coni. In type C the LV OT opens into the subpulmonary conus. In distinction from type C, type DC has an auxiliary defect in the sinus part of the interventricular septum (IVS). Type D is characterized by complete obturation of the exit and the absence of a defect in the sinus part of the IVS (type D1: OAPA RV with an intact IVS) or with a sinus defect in the IVS-DIVS (type D2: OAPA RV with noncommitted DIVS). According to the presence or absence of stenosis of the pulmonary artery (PA) or aorta, 4 subtypes are distinguished: (1) without stenosis; (2) with stenosis of PA; (3) with aortic stenosis; (4) with combined aortic and pulmonary stenosis.