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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 10  |  Issue : 1  |  Page : 51-53
Inhaled prostacyclin for the management of pneumonia in a patient with cyanotic heart disease with superior cavo-pulmonary connection

Department of Anesthesiology and Critical Care Cardiothoracic Section, Department of Surgery, Cardiothoracic Division, Department of Anesthesiology and Critical Care Hospital of the University of Pennsylvania., USA

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How to cite this article:
Augoustides JG, Abdullah I, Pochettino A, Hanson C W. Inhaled prostacyclin for the management of pneumonia in a patient with cyanotic heart disease with superior cavo-pulmonary connection. Ann Card Anaesth 2007;10:51-3

How to cite this URL:
Augoustides JG, Abdullah I, Pochettino A, Hanson C W. Inhaled prostacyclin for the management of pneumonia in a patient with cyanotic heart disease with superior cavo-pulmonary connection. Ann Card Anaesth [serial online] 2007 [cited 2022 Dec 6];10:51-3. Available from:

   Introduction Top

Inhaled prostacyclin (iPGI 2 ) is an effective selective pulmonary vasodilator. [1],[2] It not only ameliorates severe pulmonary hypertension, but also life-threatening hypoxaemia. [3] Furthermore, selective pulmonary vasodilatation with nitric oxide has reported benefit in the management of the paediatric Fontan circulation. [4],[5] The combination of inhaled nitric oxide and intravenous prostaglandin may be synergistic in preservation of pulmonary perfusion in the Fontan circulation. [6] Oral therapy with beraprost (a prostacyclin analogue) has been reported to reduce pulmonary vascular resistance as a preoperative intervention in candidates for a Fontan procedure. [7]

We report the successful application of iPGI 2 for amelioration of severe hypoxaemia in an adult with a superior cavopulmonary connection. To our knowledge, this is the first report of iPGI 2 use in this clinical scenario.

   Case report Top

A 43-year-old man sustained fractures of the cervical spine at multiple levels in a motor vehicle accident. He was transported to our intensive care unit from a referral hospital with super-added ventilator-associated pneumonia and sepsis. His history included corrected transposition of the great arteries (CTGA), severe pulmonary outflow tract obstruction, morphological left ventricular hypoplasia and morphological systemic right ventricular hypertrophy. Previous surgical management of this condition included an initial Blalock-Taussig shunt followed five years later by a Glenn procedure. The anatomy of his heart and great vessels is summarized in [Figure 1].

On admission to the intensive care unit, his blood pressure was 88/45 mm Hg. The superior vena caval pressure of 18 mm Hg (transduced via a catheter in the right internal jugular vein), and the inferior vena caval pressure was 11 mm Hg (transduced from a catheter in the right femoral vein). The oxygen saturation was 75%, despite mechanical ventilation with 100% oxygen. Sedation and neuromuscular blockers were administered to aid mechanical ventilation. The vasoactive infusions included noradrenaline and vasopressin. His head and neck were fixed in a halo and he had brisk carotid pulses with no bruits. There was a systolic precordial murmur and lungs had bilateral crackles. The abdomen was non-distended and soft. His extremities were cyanosed and clubbed. The laboratory studies were significant for polycythaemia (haemoglobin 19.4 g/dL) and acute renal insufficiency with a serum creatinine of 3.4 mg/dL (from a baseline of 1.1 mg/dL). Transthoracic echocardiography confirmed the anatomy shown in [Figure 1]. There was right ventricular (systemic ventricle) hypertrophy with normal systolic function.

The medical management in our intensive care unit consisted of the following: (a) sedation and paralysis; (b) mechanical ventilation (goal arterial oxygen tension of 35 -40 mm Hg; goal arterial carbon dioxide tension of 30-35 mm Hg); (c) culture-directed antibiotic therapy; (d) intravascular volume expansion to maintain a superior vena caval pressure of 15-20 mm Hg; (e) packed red blood cell transfusion to maintain a haemoglobin concentration of at least 16 g/dL; and, (f) maintenance of systemic vascular resistance and cardiac output with a mean arterial pressure of at least 50 mm Hg.

Selective pulmonary vasodilatation with iPGI 2 (dose of 50 ng/Kg/minute) was initiated immediately via a standard nebulizer and inspiratory limb of the mechanical ventilator to increase transpulmonary blood flow, and improve pulmonary ventilation-perfusion matching. The addition of iPGI 2 facilitated the gradual reduction of the inspired oxygen concentration from 100% to 60% over a period of 2 hours. This oxygen wean was monitored not only by continuous pulse oximetry but also by serial arterial blood gas analysis every 45 min to maintain the goal arterial oxygen tension of 35 - 40 mm Hg. Mechanical ventilation was managed to keep mean airway pressure less than 30 mm Hg. By the end of first week, the patient was weaned off all vasopressors and neuromuscular blockade. By the end of second week, the patient underwent tracheostomy and gradual withdrawal of all sedation. By the end of third week, the gradual wean of iPGI 2 was completed. The remaining hospital stay was characterized by gradual resolution of sepsis, pneumonia and renal insufficiency. He was subsequently transferred to a rehabilitation centre. The patient did not undergo any cardiac surgical procedures during this hospitalization.

Subsequently, after complete convalescence, he underwent a successful inferior cavopulmonary connection (extracardiac vascular graft from the inferior vena cava to pulmonary artery) such that all his systemic venous blood was diverted to the pulmonary circulation.

   Discussion Top

The number of patients with congenital heart disease who survive to adulthood is increasing due to advances in diagnosis and management. [8] They may present to the anaesthesiologist for problems specific to their abnormal circulation and/or with concomitant diagnoses in which the management is complicated by their pre-existing cardiopulmonary abnormalities. It is imperative that the anaesthesiologist intimately understands the unique pathophysiology inherent in each scenario.

This patient had complex cyanotic congenital heart disease with surgical palliation [Figure 1]. He presented with CTGA, which accounts for less than 1% of congenital heart disease. There is both atrioventricular and ventriculoarterial discordance. The right atrium empties into a morphological left ventricle (pulmonary ventricle) that in turn contracts into the pulmonary artery. The left atrium drains into a morphological right ventricle (systemic ventricle) that in turn ejects into the aorta. In this case, there coexisted a ventricular septal defect and pulmonary outflow tract obstruction. Due to the associated CTGA, the right ventricle was the systemic ventricle. Although CTGA is typically acyanotic, in this case it was associated with cyanosis due to the ventricular septal defect and subpulmonary valve stenosis.

This patient's morphological left ventricle (pulmonary ventricle) was hypoplastic, giving him single ventricle pathophysiology, and as such, required staged procedures to augment pulmonary blood flow. The initial procedure was a right subclavian artery to pulmonary artery anastomosis (Blalock-Taussig shunt). [8] The subsequent procedure was a superior vena caval to pulmonary artery anastomosis (Glenn procedure). [9] The third procedure was performed after his convalescence, namely a vascular graft from the inferior vena cava to the pulmonary artery with a fenestration to the right atrium. This was a completion procedure to yield a full fenestrated Fontan circulation, namely a complete cavopulmonary connection. [10]

With this patient's superior cavopulmonary connection, the transpulmonary blood flow was passive with minimal ventricular augmentation. Hence, oxygenation in this scenario was improved by interventions that achieve one or more of the following goals:

  1. Increase transpulmonary flow by elevating superior vena caval pressure (hypervolaemia) and minimizing left atrial pressure (optimize cardiac output);
  2. Decreased pulmonary vascular resistance, as by hyperventilation and resulting hypocapnia or by treatment with a direct pulmonary vasodilator such as iPGI 2 ;
  3. Enhanced ventilation-perfusion matching in the lung, also achieved with administration of iPGI 2 ;
  4. High haemoglobin concentration to improve oxygen carrying capacity;
  5. Enhanced alveolar oxygen content, which will both increase oxygen saturation and potentially increase mixed venous oxygen saturation and thereby decrease hypoxic pulmonary vasoconstriction.

This case report highlights the role of adjunctive iPGI 2 in the management of an adult with acute lung disease in the setting of complex congenital cyanotic heart disease palliated with a superior cavopulmonary connection. It was part of an integrated plan to maximize transpulmonary blood flow and gas exchange in an adult cyanotic circulation, as explained in the above mentioned goals. A further advantage is that iPGI 2 has no associated systemic vasodilatation due to its short half-life. [1],[2] This was important in this case due to already low systemic vascular resistance from sepsis.

To our knowledge, this is the first report of adjunctive iPGI 2 for effective cardiorespiratory management in the setting of cyanotic heart disease and a superior cavopulmonary connection. We anticipate that this application will become increasingly common as more Fontan patients survive to adulthood.

   References Top

1.Lowson SM. Inhaled alternatives to nitric oxide. Anesthesiology 2002; 96: 1504-1513.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Augoustides JG, Ochroch EA. Inhaled selective pulmonary vasodilators. Int Anesthesiol Clin 2005; 43: 101­-114.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Reily DJ, Tollok E, Mallitz K, et al. Successful areomedical transport using inhaled prostacyclin for a patient with life-threatening hypoxemia. Chest 2004; 125: 1579-1581.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Yoshimura N, Yamaguchi M, Oka S, et al. Inhaled nitric oxide therapy after Fontan-type operations. Surg Today 2005; 35: 31-35.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Knez I, Dacar D, Machler H, et al. The influence of different strategies on clinical outcome in patients undergoing total cavopulmonary connection. Thorac Cardiovasc Surg 1999; 47: 101-105.  Back to cited text no. 5    
6.Miyaji K, Nagata N, Miyamoto T, et al. Combined therapy with inhaled nitric oxide and intravenous epoprostenol (prostacyclin) for critical pulmonary perfusion after the Fontan procedure. J Thorac Cardiovasc Surg 2003; 125: 437-439.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Takahashi K, Mori Y, Yamamura H, et al. Effect of beraprost sodium on pulmonary vascular resistance in candidates for a Fontan procedure: a preliminary study. Pediatr Int 2003; 45: 671-675. Russell IA, Rouie-Rapp K, Stratmann G, et al. Congenital heart disease in the adult: a review with internet­accessible transesophageal echocardiographic images. Anesth Analg 2006; 102: 694-723.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Trusler GA, Williams WG, Cohen AJ, et al. William Glenn lecture. The cavopulmonary shunt: evolution of a concept. Circulation 1990; 82: IV 131-138.  Back to cited text no. 8    
9.Stumper O, Sutherland GR, Geuskens R, et al. Transesophageal echocardiography in evaluation and management after a Fontan procedure. J Am Coll Cardiol 1991; 17: 1152-1160.  Back to cited text no. 9    

Correspondence Address:
John G Augoustides
Department of Anesthesiology and Critical Care Cardiothoracic Section, 680 Dulles, Hospital of the University of Pennsylvania, Philadelphia PA, 19104-4283.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.37925

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  [Figure 1]