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Tempe DK, Datt V, Datta D. Bosentan for the treatment of portopulmonary hypertension. Ann Cardiac Anaesth 2008;11:139. 2. Colle I, Van Steenkiste C, Geerts ...
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Letters to the Editor 5.

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Hoeper MM, Halank M, Marx C, Hoeffken G, Seyfarth HJ, Schauer J, et al. Bosentan therapy for portopulmonary hypertension. Eur Respir J 2005;25:502-8 Hoeper MM, Seyfarth HJ, Hoeffken G, Wirtz H, Spiekerkoetter E, Pletz MW, et al. Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 2007;30:1096-102. Austin MJ, McDougall NI, Wendon JA, Sizer E, Knisely AS, Rela M, et al. Safety and efficacy of combined use of sildenafil, bosentan and iloprost before and after liver transplantation in severe portopulmonary hypertension. Liver Transpl 2008;14:287-91.

Authors’ reply The Editor, The readers of our article have highlighted another emerging indication of bosentan therapy. [1] Portopulmonary hypertension (PPHT) occurs in 2% to 8% of the patients with cirrhosis. Imbalance between vasodilating (decreased pulmonary expression of eNOS and prostacyclin I2) and vasoconstrictive agents (increased expression of endothelin[ET]-1 and angiotensin 1) may be responsible for misguided angiogenesis and pulmonary hypertension. Although prostacyclin analogues are efficacious, adverse effects in terms of safety, tolerability, and drug delivery occur. Bosentan could perhaps be the therapy of choice for patients with PPHT because it not only decreases pulmonary hypertension but also diminishes portal hypertension.[2] Adding further to what the readers have mentioned in their letter, I can confirm that recently, low-dose bosentan therapy (initially twice, 31.25 mg/ day; and then, 62.5 mg/day) has also been reported to be effective for the treatment of portopulmonary hypertension and renal insufficiency in a patient with Child C cirrhosis as well.[3] However, it is important to remember that presently there are few data on effects of long-term bosentan treatment for this new indication. The available evidence contributes to a building sense of excitement that bosentan may be an effective treatment in PPHT. However, the safety and dosage of bosentan for this new indication has still not been established. Moreover, bosentan therapy for this novel indication will require continuous exposure to possibly larger doses, suggesting that adverse effects may be more widespread, especially given that the ET receptors are distributed in a variety of tissues. Because so many novel therapies in the past have not lived up to their initial promise, we should protect our patients (and ourselves) and refrain from empirically administering bosentan for this emerging indication at present. Perhaps it will be prudent to wait for the results 140

of a rigorous, blinded, placebo-controlled, multicentre randomised clinical trial confirming efficacy and safety of long-term bosentan use for treatment of PPHT before routinely prescribing it for all patients with cirrhosis.

Shahzad G Raja Department of Cardiothoracic Surgery, HareÞeld Hospital, Hill End Road, HareÞeld, Middlesex UB9 6JH, United Kingdom E-mail: [email protected]

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Tempe DK, Datt V, Datta D. Bosentan for the treatment of portopulmonary hypertension. Ann Cardiac Anaesth 2008;11:139. Colle I, Van Steenkiste C, Geerts A, Van Vlierberghe H. Hepatopulmonary syndrome and portopulmonary hypertension: whats new? Acta Gastroenterol Belg 2007;70:203-9. Barth F, Gerber PJ, Reichen J, Dufour JF, Nicod LP. Efficiency and safety of bosentan in child C cirrhosis with portopulmonary hypertension and renal insufficiency. Eur J Gastroenterol Hepatol 2006;18:1117-9.

Transoesophageal echocardiography during surgery for atrial septal defect with partial anomalous pulmonary venous connection The Editor, A 24-year-old female patient was diagnosed to have a 26mm ostium secundum atrial septal defect (ASD) and mild pulmonary hypertension on preoperative transthoracic echocardiography (TTE). As TTE failed to identify the course of right lower pulmonary vein (RLPV), the patient was subjected to angiography to exclude presence of Scimitar syndrome (hypoplasia of the right lung and right pulmonary artery; anomalous drainage of right pulmonary veins; and anomalous arterial supply of the right lower lobe from the abdominal aorta). It revealed RLPV draining into hepatic vein; absence of communication between RLPV and left atrium (LA); and normal drainage of other pulmonary veins. Hence she was scheduled for surgical closure of ASD, disconnection of RLPV from hepatic vein, and its direct anastomosis to LA. After induction of anaesthesia, transoesophageal echocardiography (TOE) examination was performed, which showed opening of RLPV into inferior vena Annals of Cardiac Anaesthesia ! Vol. 11:2 ! July-Dec-2008

[Downloaded free from http://www.annals.in on Thursday, April 5, 2018, IP: 213.21.42.33] Letters to the Editor

Figure 1: Advancing the TOE probe from bicaval view shows opening of RLPV and hepatic vein inside the inferior vena cava.

(RA) junction was incised to visualize the RLPV and hepatic vein openings. Both veins were found opening separately inside the IVC. A tanned pericardial patch was used to reroute the RLPV drainage into LA through ASD, which closed ASD as well. The hepatic vein and IVC continued to drain into the RA. TOE navigation after termination of cardiopulmonary bypass showed adequate flow in the rerouted RLPV, hepatic vein [Figure 2], and IVC [Figure 3]. Ten percent to 15% of ostium secundum atrial septal defects are associated with partial anomalous pulmonary venous connections (PAPVC). TOE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence surgical management.[1] Pericardial patch may obstruct the flow of rerouted pulmonary vein and IVC, which should be ruled out after weaning the patient from CPB.

Shrinivas Gadhinglajkar, Rupa Sreedhar Department of Anaesthesiology Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India Address for correspondence: Dr. Shrinivas Gadhinglajkar, Department of Anaesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, Kerala, India. E-mail: [email protected]

REFERENCE 1.

Figure 2: Inspection of inferior vena cava at the level of hepatic vein by advancing the probe from bicaval view and changing the sector angle to 70-80°. It reveals the pericardial patch; and unobstructed ßow through RLPV, hepatic vein, and IVC.

Ammash NM, Seward JB, Warnes CA, Connolly HM, O’Leary PW, Danielson GK. Partial anomalous pulmonary venous connection: Diagnosis by transesophageal echocardiography. J Am Coll Cardiol 1997;29:1351-8.

Transoesophageal echocardiography during Senning’s operation The Editor,

Figure 3: ModiÞed bicaval view revealing patch at the inferior vena cava–right atrial junction, with separate ßow via pulmonary vein and IVC

cava (IVC) 1 cm proximal to hepatic venous opening [Figure 1]. This finding was also confirmed by the surgeon after sternotomy. Hence direct anastomosis of RLPV to LA was deferred. On CPB with deep hypothermic circulatory arrest, the IVC-right atrium Annals of Cardiac Anaesthesia ! Vol. 11:2 ! July-Dec-2008

A 3-year-old male, diagnosed as a case of dextroposed transposition of great arteries (d-TGA) preoperatively, underwent Senning’s operation. TOE examination performed after induction of anaesthesia (using Philips Sonos 7500, USA) revealed pulmonary artery (PA) positioned posterior to the aorta [Figure 1] and ventriculoarterial discordance [right ventricle (RV) draining into the aorta and left ventricle (LV) draining into the PA] [Figure 2]. Dynamic left ventricular outflow tract obstruction (LVOTO) was present. Other features were enlarged right atrium and RV; intact interventricular septum (IVS); atrial septal defect (ASD) measuring 141

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